Cardiology and Nephrology

Table of contents :
Cover
Cariology
Content
o Cardiac cycle
o Neck veins
o Symptomatology
o Heart Failure
o Acute Pulmonary Edema
o Diseases Of Pericardium
o Diseases Of Myocardium: Myocarditis & Cardiomyopathy
o Rheumatic Fever
o Infective Endocarditis
o Coronary Artery Disease : Angina pectoris & Myocardial infarction
o Dyslipidemia
o Pulmonary Hypertension
o Pulmonary Embolism
o Systemic Hypertension
o Valvular heart diseases
o Congenital heart diseases
o Arrhythmias
o Sudden cardiac death
o Diseases of aorta: Aortic Aneurysm & Dissecting Aortic Aneurysm
o Peripheral arterial diseases
o Shock
o Heart Transplantation
Nephrology
Contents
o Introduction
o Symptomatology
o GlomeruIonephritis
o Nephrotic syndrome & Nephritic syndrome
o Tubulointerstitial nephropathy
o Pyelonephritis
o Renal vascular diseases
o Renal failure
o Renal replacement therapy
o Renal transplantation
o Hereditary renal diseases
o Electrolyte & acid base imbalance

Citation preview

w

'd

Malhsuismdl Ss^aiaaii Kasr Al-Ainy School of Medicine

Cairo University ‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

By Mahmoud Sewilam

Kasr Al-Ainy School of Medicine Cairo University

First Edition

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Cardiology Topic

Page

o Cardiac cycle

1

o Neck veins

4

o Symptomatology

5

o Heart Failure

15

o Acute Pulmonary Edema

31

o Diseases Of Pericardium

33

o Diseases Of Myocardium: Myocarditis & Cardiomyopathy

39

o Rheumatic Fever

44

o Infective Endocarditis

48

o Coronary Artery Disease : Angina pectoris & Myocardial

55

infarction

o Dyslipidemia

65

o Pulmonary Hypertension

67

o Pulmonary Embolism

69

o Systemic Hypertension

72

o Valvular heart diseases

80

o Congenital heart diseases

95

o Arrhythmias

104

o Sudden cardiac death

118

o Diseases of aorta: Aortic Aneurysm & Dissecting Aortic Aneurysm o Peripheral arterial diseases

120 125

o Shock

127

o Heart Transplantation

131

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Cardiac cycle

n SI

Systole

n S2

Diastole

SI

Systole

I. Systole: 1. First heart sound :

The cardiac cycle start by contraction of ventricles resulting in closure of mitral & tricuspid valves producing the first heart

sound (SI) 2. Isometric contraction phase:

•

II. Diastole:

4. Second heart sound:

• After evacuation of blood, the ventricles relax, so the pressure in the aorta and pulmonary artery will exceed the pressure in the ventricles resulting in closure of aortic & pulmonary valves producing the second heart sound(82) 5. Isometric relaxation phase:

The ventricles continue to contract while the

The ventricles continue to relax while four valves of the

four valves of the heart are closed, so the

heart are closed, so pressure inside the ventricles falls rapidly without change in the volume.

pressure inside the ventricles rises rapidly

without change in the volume 3. Ejection phase:

When the pressure in the ventricles exceeds the pressure in the aorta & pulmonary artery, the aortic & pulmonary valves will open (normally with no sound) Then the blood rushes from the ventricles to

the aorta and pulmonary artery,

First rapidly: maximum ejection phase

6. Ventricular filling phase:

When the pressure in the ventricles becomes lower than the pressure in the atria, blood flows to the ventricles passively, First rapidly : maximum filling phase Then slowly:reduced filling phase 7. Atrial systole:

• The last amount of blood in the atria is pushed actively by atrial contraction in the late diastole.

Then slowly: reduced ejection phase 8. Ventricular contraction: occurs again & the cycle is repeated.

N.B.: any change in heart rate ,is a change in diastole, systole is constant: Tachycardia shortens diastole while bradycardia lengthens diastole.

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Pulmonary valve

Heart Sounds and their relation to cardiac cycle:

Aortic valve

Right coronary a.

1. II.

Heart sounds(S1,S2,S3,S4) Additional heart sounds (Systolic clicks & opening snap)

III.

coronary a

Murmurs Tricuspid

Heart sounds

n:

Mitr^ valve

Coronary sinus

Posterior inlenrentricuiar

branch of right coronary a

a o

••C u V

w"

S3

S2

SI

Systole

S4

Systole

Diastole

2. Second heart sound, S2

1. l irst heart sound, SI

❖ Time: ■ Beginning of diastole ❖ Caused by :

1 1 ■

SI

Beginning of systole

■

■

Valvular: closure of atrioventricular (mitral &

■

tricuspid) valves Muscular: early ventricular contraction (vibrations of chordae tendineae & papillary muscles)

■

At the apex of the heart

I | 1

Valvular: Closure of semilunar (aortic & pulmonary) valves:

o Over aortic area : S2 is single(A2 only heard) o Over pulmonary area : S2 is split(A2 followed by P2)

❖ Site of maximum intensity : ■

I

Over base of the heart

❖ Intensity:

I ®" Causes of Accentuated 82:

®" Causes of Accentuated SI: 1. MS-TS

2. Systemic hypertension

1. t A2 in systemic hypertension 2. t P2 in pulmonary hypertension Causes of Weak (muffled)82: 1. iA2inA8«&AR

®" Causes of Weak (muffled) SI: 1. MR-TR

2. iP2inP8&PR 4. Fourth heart sound,84

2. Calcified MS

3. Third heart sounds, S3

♦♦♦ Time:

■

Early diastole sound heard shortly after S2 best

■

heard by the cone in left lateral position.

Late diastole (presystolic)sound heard just before 81 best heard by the cone in left lateral position.

"> Mechanism:

■

■

Excess ventricular vibrations (blood gush in ventricle after opening of atrioventricular valve)

1

Forcible atrial contraction against high ventricular end diastolic pressure

Causes of the sound: 1. Normal in children & young adult

1. Normal in elderly person

2. Volume overload in:

2. Pressure overload in :

o o o 3.

o LV: systemic hypertension, AS o RV: pulmonary hypertension, PS

LV; MR,AR, VSD RV:TR,PR, ASD Hyperkinetic circulation Flabby myocardium in:

o LVF (apical gallop) o RVF (tricuspid gallop) o Dilated cardiomyopathy

3. Reduced compliance of ventricles in: o

Myocardial infarction

o Restrictive & Hypertrophic cardiomyopathy

❖ N.B.: Callop rhythm (triple rhythm): (S3 or S4 or Both)+ tachycardia

A. Protodiastohc (ventricular) gallop = S3 + tachycardia B. Presystolic (atrial) gallop = S4 + tachycardia C. Summation gallop = S3 + S4 + tachycardia e.g. hypertensive heart failure

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

I

Expiration Normal

Inspiration

Jl

Ir

S2

S2

Normally : left ventricle ejection period is shorter than that of right ventricle ,so the pressure closing aortic valve is higher than that closing pulmonary valve so aortic valve closes before pulmonary valve I.e. A2 precedes P2 Relation of splitting to respiration:

S Splitting Is Only Detected Over Pulmonary Area : Pulmonary component is weak & heard over pulmonary area only while aortic component is loud heard at both A & P areas.

During inspiration : t Venous return will increase RV Load, with delayed closure of pulmonary valve. Meanwhile the lung is expanded & retains more blood in its vessels with decrease in LV load, and so earlier closure of aortic valve.

So during inspiration —* wide splitting of S2 During expiration: the reverse occurs —> narrow splitting or fusion (single S2) Causes of:

Caused by functional(physiological) abnormality due to : A. Increases the blood flow through a structurally normal heart e.g. hyperdynamic circulation B. Flow into dilated chamber e.g. •

Ventricular dilatation in HP

• Aortic or pulmonary dilatation in systemic or P.HTN

o May propagate to other areas o Associated with thrill + symptoms & signs

o

Localized

o No thrill - no symptoms nor signs

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Neck veins Introduction :

Internaljugular vein is valveless i.e. has no venous valves: so it's direct continuous with the right atrium which acts as manometer which reflects exactly the changes in right atrium

Right IJV is preferred as left sided ones may be present falsely prominent(high) as the left innominate vein (Brachio-cephalic) may be compressed by the arch of aorta.

Normal neck veins: pulsating not congested, empty with inspiration with systolic collapse

Physiology of the waves:

o Normal Jugular venous pressure (JVP)= 0-5 cmH20

o Since right atrium is 5 cm below the sternal angle. o o

■■Hyb

So Central Venous Pressure (GYP) = JVP + 5 cm = ... cmH20 {Normal CVP = 7-12 cmH20 } Consists of 3 positive (A,C & V) & 2 negatives (X & Y)

IVENTRICU ayeioiB

istss SYSTOLE

DIASTO

ATRIAL syntote diasloie

•I

a wave

C wave

Wave ■

a wave

o

X descent

y descent

V wave

❖ Represents Right atrial contraction (atrial systole)

Timing •

Diastolic

(Presystolic) wave ■

c wave

o

Upward bulge of tricuspid valve into right atrium at start of ventricular contraction or transmitted from adjacent carotids

■

X descent

o

■

V wave

o

at the onset of ventricular systole Right atrial relaxation (normal systolic collapse) Right atrial filling from the venous blood

■

y descent

o

Right atrial emptying to right ventricle (diastolic collapse)

•

Systolic wave

• •

Systolic wave Systolic wave

•

Diastolic wave

• Possibilities after measurement:

a) Normally the JVP is visible 2-4 cm above the sternal angle i.e. not congested

b) Abnormally: the upper border of the IJV pulsation > 4 cm i.e. congested; comment on the following : 1. Congested Pulsating neck vein cm above angle of Lewis 2. Relation to respiration:

o Either empty with inspiration or inspiratory filling. 3. Hepato-jugular reflux or abdomino-jugular reflux or one minute abdominal compression test: -i-ve or -ve

o Compression of right hypochondrium for 1 minute -+ engorged neck veins(|JVP > 3 cm for 30 seconds) due to painful reflex contraction of abdominal wall.

4. Timing:either systolic collapse or systolic expansion • Radial pulsation is preferred for timing of the venous pulsation

• All causes are systolic collapse except { AF,TR & cannon a waves^ Systolic expansion }

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Causes of congested neek veins:

o

A. Pulsating: f Right atrial Pressure : right sided HP, TR

B. Non pulsating : can't see upper border

o Severe right sided failure:severe congested neck veins

o

o o

t intrathoracic Pressure: physiological(cough, straining), pneumothorax, emphysema

o Complete SVC obstruction :thrombosis & mediastinal

1 Intraabdominal Pressure : tense ascites t Blood volume: anemia, pregnancy, fluid

o Pericardial diseases:pericardial effusion & constrictive pericarditis

syndrome

overload

❖ Common abnormalities in jugular venous pulse = abnormal neck veins waves 2. Giant(prominent)

1. Absent a wave in:

a wave (forceful atrial contraction) in :

3. Cannon a waves(synchronous atrial & ventricular contraction)in: K

vw

A

JUA

Y

o

AF(no atrial contraction)

o

IS

o

PS

Regular: o Nodal rhythm o Paroxysmal nodal tachycardia Occasionally:Atrio-ventricular dissociation:

o Pulmonary hypertension

o Paroxysmal ventricular tachycardia o

Complete heart block

4. Causes of giant v wave or causes of obliteration of x descent? Systolic expansion; AF,TR V

i >

6. Prominent y descent in:

5. Prominent x descent in:

7. Attenuated or absent y descent in :


i Peripheral resistance & f RBF & UOP

beta dose :

o 3-10 pg/kg/min

o >10 pg/kg/min

o

o

Positive

inotropic & chrontropic —> f

Generalized VC

-♦tSBP

%

o Stimulate pi receptors and weak a stimulant

o 2.5 - ID pg/kg/min o Positive inotropic more than

chrontropic

COP & SBP

sodium excretion

c. Phosphodiesterase III inhibitors; inodilator i.e. positive inotropic & peripheral VD; 1. E.g. amrinone, milrinone, enoximone 2.

Mechanism of action:

Adenyl Cyclase ATP

Phosphodiesterase cAMP

C

Positive inotropic

AMP

Mixed VD

o Inhibit phosphodiesterase —cAMP in heart and blood vessels —> direct myocardial stimulant and smooth muscle relaxation ^ mixed VD (artery & vein) -> j preload & J. afterload 3.

Side effects:

•

t Myocardial oxygen consumption and so worsens the angina pectoris .

• •

Thrombocytopenia Hepatotoxicity

d. Cardiac glycoside : Digitalis e.

Calcium sensitizer : levosimendan

27

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Cardiac glycoside: Digitalis Mechanism of action action:

J

By partial inhibition of Na+/K+ATPase enzyme -»• t intracellular Na'^ -> i transmembranal exchange of extracellular Na^ & intracellular Ca^ —> f intraceiiniar Ca'*^ -+ sliding of actin & myosin K inhibit action of digitalis on ATPase, so hypokalemia —> digitalis toxicity.

the contractility

Action;

Myocyte

Sinoatnal Node

0

I Conductivity

•>2K+ Atrtoventricular

Node(AVN) 3Na+ OIMH'-N.

Na+

Bundle of His

^Na+

Purt(ln}e

E

Sarcoplasmic

Ca++- In digitalis toxicity dijferent arrhythmia may occur. 5. Diuretic effect: fCOP -+ t renal blood flow —> fUOP -> i edema. 6.

ECG:

•

i Conduction : long PR interval

•

Sagging depression of ST segment, flat or inverted T wave.

*1* Indications: A. Heart failure

B. Rapid ATRIAL arrhythmias e.g. AF, atrial flutter, paroxysmal atrial tachycardia • I.

Contraindications: Relative CI:

. Block

1. Hypertrophic cardiomyopathy 2. Partial HB may change into complete HB 3. AY (junctional or nodal) rhythm: HR will decrease II.

Absolute CI:

1. Ventricular tachycardia may change into VF 2. Digitalis toxicity

28

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

fUOP

j, pigitalization of t:he patienti B. Digitoxin o Metabolized mainly hepatic o Excreted mainly renal & bile o Oral tablet = 0.1 mg

A. Digioxin (lanoxin) o Metabolized mainly renal o Excreted mainly renal

o Oral tablets = 0.25mg

1(9^

0 IV ampules = 0.5 mg

l'

❖ Dosage : I. Loading method (rapid digitalization) over 24 hours: a. Loading dose : o 0.25 mg - 0.5 mg orally or IV over 30 minutes followed by 0.25 mg orally or IV / 6 hours for 2 -3 doses,

a) Loading dose: o Given orally 0.2-0.3mg /6 hours for 4 doses

b) Maintenance dose:

h. Maintenance dose:

o 0.125mg - 0.5 mg /day orally • N.B.: IV digitalization is indicated in acute HE,Rapid atrial arrhythmias. H.

o O.OSmg -0.2 mg /day

Non Loading method (cumulative method): start by the maintenance dose.

Signs of adequate digitalization: therapeutic serum level 0.5 -2 ng/ml 1. Improvement of manifestation of HF 2. Decrease hear rate(70-80 b/m)

❖ Digitalis toxicity^ ❖ To avoid digitalis toxicity : o Decrease the dose (give half the dose) o Drug holiday ❖ Signs of digitalis toxicity : toxic serum level > 2.5 ng/ml 1. 1.

Factors enhancing toxicity: Electrolytes: Hypokalemia, alkalosis & Hypomagnesaemia Hvpercalcemia

4. Doses:

*

_

Repeated loading doses Large maintenance doses Drugs e.g.

Hypoxia & acidosis 2.

Endocrine & metabolic:

^

CCB, Corticosteroids

Hypo or hyperthyroidism, Hepatic & renal diseases

K loosing Diuretics

Extremes of ages H.

Clinical picture of toxicity: 1. CNS

2. CVS

Blurring of vision

A. Early: Bradycardia < 60 b/m

Colored vision:

B. Late :

yellow & green Confusion,

•

Delusion, Delirium Headache,

3. GIT

4. Others

THE FIRST

Gynecomastia

SYMPTOM

• Heart Block : partial then complete

Convulsion

•

Arrhvthmia (atrial & ventricular):

• Anorexia, nausea

& vomiting

1) Tachycardia 2) Flutter & Fibrillation

3) Etrasystoles(pulsus bigeminus, pulsus trigiminus)

Hallucination

Treatment of digitalis toxicity: ..—^

.

—

.iii.iia

1) Stop digitalis

2) Correct any factor enhancing toxicity e.g.: o Correct hypokalemia by stopping K loosing diuretics & giving potassium orally or IV 3) Digitalis specific antibodies (digoxin immune Fab IV). 4) Symptomatic treatment:

A. For sinus bradycardyia or heart block : give atropine

B. For ventricular arrhythmias : give phenytoin, lidocaine & avoid DC shock except for VF. C. For vomiting : give metoclopramide . 29

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

B. Cardiac assist devices:

a) Intraaortic balloon counterpulsation maintain hemodynamic stability:

o Catheter with a balloon is inserted into femoral artery till it reaches descending aorta under X-ray o Balloon in rhythmically inflated during diastole -+ f diastolic pressure in aorta —*■ f perfusion pressure in the proximal aorta and coronary arteries and deflated during systole —> j afterload. o Used temporarily also in severe LVF awaiting for surgery e.g. in severe MR or acute VSD b) Implantable cardiovertcr defibrillator (ICD) :

c) Cardiac rcs> nchroni/ation therapy (CRT) b> Bhentricular pacing or L\' pacing

Implantable cardioverter-

■M^^^^^Bfcdefibrillalof

❖ Benefit:

1 • For primary prevention of sustained ventricular tachycardia that cause sudden cardiac death

1

I

• Improve the cardiac performance and reverse the ventricular remodeling

Indications: o

NYHA class 11 to III

o Systolic dysfunction LVEF < 35 %. o Cardiomyopathy.

o

NYHA class 111 to IV despite proper treatment

o Systolic dysfunction LVEF < 35 %. o Cardiomyopathy with intraventricular conduction delay or bundle branch block as evidenced by wide QRS >0.12 sec in EGG Diseased heart

Donated heart

removed

transplanted In recipient

C. Cardiac transplantation: Indications : o o

For young patients with severe resistant HF. Severe systolic dysfunction(EF = 10- 20 %)despite proper treatment. Contraindications: irreversible pulmonary hypertension.

30

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Acute Pulmonary Oedema(APO)

❖ Derinition: rapid accumulation of fluid in the interstitial & intra-aiveolar spaces of the lung. ❖ Etiology: II. 1.

Cardiogenic Pulmonary Oedema = Acute Left Side Heart Failure.

• Sudden increase in the pulmonary venous pressure.

»

Non Cardiogenic Pulmonary Oedema = Acute Respiratory Distress Syndrome(ARDS)

Sudden increase in the pulmonary capillary permeability

1. Acute left sided heart failure e.g. Acute myocardial infarction

1) Pulmonary: o Aspiration of gastric contents,

2. Acute exacerbation of chronic LSHF; e.g. MS with a precipitating factor as AF.

o Pneumonia, lung Contusion, o Inhalation of toxic gases e.g. sulfur Dioxide

o Sudden Expansion of collapsed lungs e.g. rapid aspiration of massive pleural effusion 2) Extra-Pulmonary;

• Pulmonary capillary wedge pressure: a. > 20 mmHg ^ Interstitial edema. b. > 25 mmHg —>• Alveolar edema.

Amniotic fluid embolism

Clinical picture :

Severe hypoAlbuminemia Bums, Pancreatitis

A. Of the cause :

CNS: Trauma, head injuries & stroke.

• Chest pain in acute MI in Cardiogenic PC • Stroke clinical picture in Non Cardiogenic PC B. Typical clinical picture of APO :

Die

_

End organ damage: liver & renal failure.

V

1. Severe dyspnea at rest & orthopnea 2. Sweating & marked irritability. 3. Central cyanosis

Septicemia, Shock

Jk Pulmonary capillary wedge pressure < 20 mmHg

4. Cough with expectoration of excessive frothy blood-tinged sputum 5. Generalized bubbling crepitations 6. Generalized wheezes

Dilated prominent upper lobe vessels

Investigations: ma

1. Of the cause: e.g.

'

Alveolar oedema

CBat's wings')

o ECG & Cardiac enzymes for AMI(Cardiogenic PO)

..Cardiomegaly

o Brain imaging(CT scan) for Stroke(Non - Cardiogenic PO) 2. Chest X-Ray:

Kerley B lines (interstitial oedema)

o Haziness of lung fields, o Moustache sign: pulmonary congestion:

Pleural Effusion

Exaggerated pulmonary vascular markings especially in upper lung zon^ o Kerley B lines: interstitial edema, basal, thin, short lines Pulmonary edema o Bat's wing: alveolar edema 3. Arterial Blood Gases(ABG): o

PO2: decreased.

° PCO2: decreased first, then, increased lately in severe cases Treatment:

1. Treatment of the cause: e.g.

o Reperfusion (revascularization) for AMI(Cardiogenic PE) o General care, Antiplatelets for Stroke(Non - Cardiogenic PE)

2. Treatment of Non - Cardiogenic PO: mechanical ventilation with protective lung strategy & IV corticosteroids 31

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

3. Treatment of Cardiogenic pulmonary oedema: —

''t'-l-

■.

PDAs

1. Hospitalization in ICU 2. Treatment of the cause & Precipitating factors. 3. Position: Bed Rest in sitting position

4. Breathing: Oxygen administration 60- 100% via mask or ETT & mechanical ventilation if needed. 5.

Diuretics:

o IV FUROSEMIDE 40 mg to correct hypervolemia to be repeated every 'A hr until symptoms relief. •

N.B. :

a. Extracorporeal ultrafiltration : in patients who are volume overloaded when the response to diuretics is sluggish. b. Venesection (Phlebotomy): old method to decrease blood volume and venous retum . 6. IV vaso Dilators : Nitroglycerine, Na nitroprusside, Nesiritide. 7. rv Inotropes e.g. Digitalis, Dobutamine, Dopamine 8. Antagonize the sympathetic response :

o MORPHINE rv 5 mg to relieve the anxiety and decrease the sympathetic stimulation, thus causing VD —>

venous

pressure.

9. Aminophylline for bronchospasm : 250 - 500 mg FV infusion slowly 10. Assist devices e.g. intra-aortic balloon counterpulsation.

Refractory HF = Resistant HF = Intractable HF Definition : This term is used when there is poor response to usual lines oftreatment. Etiology ; 1) Persistence ofthe cause e.g. o

Aortic Regurge(digitalis fdiastole and so t regurge)

o

Idiopathic hypertrophic subaortic stenosis: digitalis t contractility and so t obstruction

2) Persistence of precipitating factors e.g. rheumatic activity 3) Improper management: inadequate rest, excess salt intake, improper drug doses 4) Severe myocardial damage(ABSOLUTE refractory HF)e.g.

Myocarditis, Cardiomyopathy & Extensive myocardial infarction Management:

1) Treatment of the cause & precipitating factors. 2) Same lines of treatment with use of alternatives drugs e.g. dobutamine instead of digitalis 3) Use of IV drug combination e.g. : o

rv diuretics (furosemide)+ IV vasodilators (Nesiritide)+ IV Inotropes(Dobutamine)

4) Extracorporeal ultrafiltration . 5) Cardiac assist devices e.g. Intraaortic balloon counterpulsation or cardiac resynchronization therapy 6) Cardiac transplantation. Diseases Of Pericardium

The heart wall composed of three layer: pericardium, myocardium,endocardium . Anatomical consideration: pericardium is formed of 2 layers separated by I0-15ml lubricant fluid: Parietal layer: fibrous, protect heart from sudden cardiac dilatation a) b) Visceral layer ❖ Diseases Of Pericardium: 1.

Acute Dry Pericarditis (AcuteFibrinous Pericarditis)

2.

Pericardial Effusion & Cardiac Tamponade Constrictive Pericarditis (Pick's Disease) Adhesive pericarditis

3. 4.

(iMMt muKw) •hownnrM

of rnyocaimim)

Endocardium

(Innor aurfaoa of myocacdkin^

32

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Inflammec

petlcardlu (pertcardt RlgMLuns

¥.

Friction rub

STE concave up Pericardia! Effusion & Cardiuc Tamponadc .

V - -''iMlnMSiil

Beck's triad

Prayer s position

Pressure

mVAt^mAIV Deep X descent, Attenuated Y descent

Expiration

Inspiration

Expiration

Puisus Paradoxus bimnor v«na civt

Tboraoc aorta

Diaphfagm H«p^ win

I

Electrical alternans

Ascites precox; Kinking of hepatic veins Obstruction of lymphatics

Constrictive Pericarditis

Square root sign

Pericardia! knock

Deep X descent, Deep Y descent

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Acute Dry Pericarditis =

Pericardia! Effusion & Cardiac Tamponade

Constrictive Pericarditis ■

Acute Fibrinous Pericarditis

Pick's Disease

Etiology :

Collection of fluid in pericardial sac due to; Hcmopcrlcardlum; Blood accumulation : Hemorrhagic blood diseases e.g. Hemophilia. Rupture heart as in trauma or MI Rupture of aortic aneurysm Rupture coronaries during catheterization Hydroperlcardlum (Transudate accumulation): All causes of generalized edema (cardiac, hepatic, renal, nutritional, allergic) Myxedema

Intlammation of the

pericardial sac due to; 1.

Idiopatllic.

2. Infection: the most common cause:

Viral; the commonest

cause e.g. Coxsackle B virus or Echo virus

Bacterial, TB, Fungal. Inflammation: FMF

latrogenic e.g. procainamide, hydralazine.

3.

5. Irradiation

6.

Immiinological: Dressler's Syndrome Post-cardiotomy Syndrome

Myxedema Myocardlal Infarction 10. Malignant Infiltration e.g. leukemia

5.

o

Asbestosis.

o

Histoplasmosis

o

Fibrinous membrane

resolution

Pathology, Pathogenesis: Cardiac tamponade = severe pericardial

Pericardium is markedly

effusion + obstructive shock

thickened & flbrosed, with marked adhesions

between its two layers,

leading to constriction of

A. 250 ml rapidly or B. 1000 -2000 ml slowly ❖ Leading to :

heart

Symptom of the cause e.g. TB toxemia General symptoms of a) interference with function of both sides of heart; SVC, PVC & low COP toxemia;fever, malaise, headache

symptoms

:

Chest pain : Cause : inflamed

Calclflcation of

pericardium is common Ventricular filling is unimpeded in early diastole but it gets reduced abruptly when the

b) Stretch of parietal layer & pressure on surroimding structures. ❖ Ventricular filling is impeded throughout

elastic limit of

diastole.

pericardium is reached in

parietal pericardium +

late diastole.

extension of inflammation

2.

to parietal pleura. Site : precordial

3. Radiation: Root of neck & shoulders

(by phrenic nerve) 4. Character:

Stitching Stretching dull aching with development of effusion.

5. Duration : continuous 6.

Symptoms: I. 11. III.

Symptoms of LCOP: see before.

1. Cause : stretch of parietal pericardium 2. Site ; precordial 3. Radiation: Shoulders 4. Character: Dull aching B. Pressure symptoms : I.

t By: lying flat & rotation iBy : sitting up and leaning forward

Symptoms of the cause e.g. TB toxemia Symptoms of systemic & pulmonary venous congestion: see HF.

IV. Local symptoms ; A. Chest pain

of trunk.

7.

Not common in

myxedema.

Myocardial infarction. Malignant infiltration Suppuratlve pericarditis(pus accumulation): Infection by pyogenic organisms e.g. staphylococcal and streptococcal infections Chylous effusion (lymph accumulation): Rupture or obstruction of thoracic duct by,

❖ Depending on time for adaptation; if accumulation of fluid in pericardial sac is either

Symptoms

Local

Never rheumatic fever

since its pathology is exudative which heals by

trauma, tumor & filariasis.

develop causing friction between two layers of pericardium.

1.

Sarcoidosis.

o

TB, Renal failure.

9.

III.

cause,

o

Hemorrhagic pericarditis is a severe form of

7. Renal failure.

II.

Same causes of dry pericarditis but add; o TB; the commonest

serous effusion containing many RBCs, and may occur in the following conditions;

Rheumatoid arthritis, SLE, Scleroderma.

I.

two layers of the pericardium due to ;

the most common cause.

Rheumatic fever.

o

Seroperlcardlum (Exudate accumulation): All causes of dry pericarditis particularly T.B Is

❖ Adhesions between the

2.

IV.

No local symptoms ;

• No Pericordial pain • No Pressure symptoms • No Prayer's position

On lung : Praver's position ; dyspnea oblige patient to lean forwards to shift fluid of pericardial effusion away from pulmonary veins and LA (posterior structure) —> J. PVC On esophagus(dysphagia). Left RLN (hoarseness), bronchi(cough). 34

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Signs: I.

Signs of the cause e.g.

3) Relation :

Signs of the cause e.g. TB II. Signs of systemic venous congestion: A. SVC: t JVP, congested NY early pulsating ,later non pulsating, with : 1. Kussmaul's sign: inspiratory filling of neck veins & drops with expiration : Failure of the right atrium to accept the increased venous return during inspiration due to cardiac compression during inspiration. 2. Gibson's sign: prominent & deep X descent A. Hepatojugular reflux : positive. 4. Attenuated or absent Y descent 4. Friedreich's sign (diastolic collapse): prominent & deep Y descent due to rapid emptying ofthe congested neck veins in a short time after opening of

• Not related to inspiration (DD pleural rub)

B. IVC:

TB

11.

General signs of toxemia

III.

Local signs:

Pericardial friction rub:

1) Cause : • Friction between 2 layers

of pericardium 2) Site: •

Over the whole

pericardium but best hard over the Bare area and the Base of the heart

4) Character: • Scratching, leathery, gritty. 5) Timing: • Systole & diastole. 6) t By: • Pressure of stethoscope, leaning foreword 7) iBy: • Development of effusion.

I.

the tricuspid valve. 1. Enlarged tender liver 2. Ascites precox: Ascites before edema due to a) Kinking of hepatic veins entering IVC causing early liver congestion & ascites

b) Obstruction of lymphatics passing through central tendon of diaphragm causes accumulation of lymph in peritoneum. III. Signs of LCOP: See before + • Pulsus paradoxus: exaggerated decrease(>10mmHg)in pulse volume during inspiration [exaggeration of normal] IV. Local Signs: IV. Local signs : A. Inspection & palpation :

A. Inspection & palpation:

1. Apical pulse : absent (invisible & impalpable)

1. Apical pulse: weak or

2. Precordial bulge in children

absent

1. Weak distant heart sound

2. No cardiac enlargement: small quiet heart

2. I breath sounds (pleural effusion)

B. Auscultation :

B. Auscultation :

C. Percussion :

1. Weak distant heart sound

1. Dullness outside apex 2. t size of bare area "dull base"

2. Pericardial knock:

3. Shifting dullness over pulmonary area (disappear on sitting) 4. Dullness on right border of sternum 5. Ewart's sign : dullness over left subscapular

diastolic shock

• Early 3rd sound • Due to sudden halting of relaxing ventricles by rigid pericardium

region due to compression of base of left lung (collapse) by effusion ❖ Beck's triad for diagnosis of cardiac tamponade: 1. Decreased BP

2. Distended venous pressure

3. Distant(muffled) heart sounds. ❖ Complications o

Pericardial effusion

1. Acute cardiac tamponade, leading to obstructive shock with severe SVC

2. Constrictive pericarditis 3. Complication of aspiration

1. Cardiac cirrhosis

2. AF in 30% ofcases due to

J, ventricular filling —»-t atrial pressure ^ atrial dilatation -+ AF.

Differential diagnosis: Other causes of acute

chest pain Other causes ofST

segment elevation e.g.

DD of cause e.g. TB,from clinical picture & investigation Constrictive pericarditis. Restrictive cardiomyopathy TR, TS, Mediastinal syndrome

AMI & Prinzemtal

Generalized edema : Congestive HF, liver cirrhosis & nephrotic syndrome. N.B.: in Congestive HF: LL edema before ascites,No Pulsus paradoxus,

angina.

different types of dyspnea, cardiomegaly with murmurs & gallop. 35

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Investigations:

1. Investigations for cause 2. Chest -X ray • To exclude pericardial effusion.

• Fluoroscopy: minimal cardiac pulsation

Small globular heart:

1. Cardiac base : Wide & broad 2. Cardiac borders:

decreased cardio-thoracic

o

Stenciled, Smooth with Symmetrical bulging

ratio

o

Double contour all around the cardiac borders.

Calcified pericardium: egg shell appearance ± Evidence of TB lung Fluoroscopy: minimal cardiac pulsation

3. Cardiophrenic angle on the left side is o

Acute

o Obtuse in lower lobe collapse (Rotch's sign) 4. Lung oligemia : decrease bronchovascular markings 5. Radiological appearance of the heart simulates a flask

CT & MRI: the most

precise technique diagnosis

in

6. If complicated pneumopericardium (area ofjet black translucency surrounding the cardiac shadow) o Elevated ST segment; concave up (saddle

shaped)

3. ECG: Low voltage with flat or inverted T wave + o Electrical aiternans: may occur due to beat to beat alteration of QRS axis due to swinging of

AF in 30 % of cases

the floating heart in the effusion 4. Echocardiography:

o To exclude pericardial effusion.

o Most sensitive test to detects pericardial effusion o Determines the severity (cardiac tamponade

causes diastolic collapse of RV)

Show pericardial thickening ± calcification Exclude effusion

Cardiac Catheterization & Angiocardiography:

not essential after use of

echocardiography.

o Show gap between tip of cardiac catheter and peripheral cardiac shadow o Angiocardiography demonstrates actual size of

Square root sign in pressure tracing of the RV and LV: diastolic dip

followed by plateau

heart inside effusion

6. Biopsy :

Pericardial biopsy : if malignancy or TB are

Endomyocardial biopsy to

suspected

exclude restrictive CM

7. Diagnostic pericardiocentesis (pericardial aspiration): to differentiate of the nature of

Exploratory

thoracotomy: diagnostic.

fluid

A. Hemopericardium: blood stained with many RBCS B & C : Transudate & exudate:

1. Aspect:

B. Transudate

C. Exudate

o

0

Colorless ,

Yellowish

Clear

Turbid

3. Cholesterol

0 3 gm/dl o > 45mg/dl

4. LDH

0

0

2. Proteins

200IU/L

5. Pericardial Fluid proteins /Serum Proteins o

0.5

6. Pericardial Fluid LDH/ Serum LDH o

< 0.6

0

7. WBCs

o

< 1000/

0 >1000/mm^

> 0.6

8. Specific gravity

o

60 mg/dl

mm^ 0

>1016

o 10 mmHg)& not a paradox. In Pericardial effusion :

DECREASED Ao FLOW INCREASED PA FLOW

AND PRESSURE

iK" INCREASED RETURN MAINTAINS PRESSURE

FALLS

FIUING FILLING

-TENSE PERICARDIAL EFFUSION

EXPIRATION

INSPIRATION

o Inability of right side of heart to accommodate V.R., meanwhile lung expand and accommodate greater amount of blood

o Accordingly the amount reaching left side decreases, there is exaggerated J.J, in SBP(> 10 mmHg)and exaggerated IJ, in pulse volume. • Detected by palpation (femoral & carotids) or by sphygmomanometer. •

Occurs also in:

A. Constrictive pericarditis B. Massive pulmonary embolism C. Status asthmaticus & COPD

37

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Adhesive pericarditis

❖ Etiology : 1. Late complication of Rheumatic fever

2. ❖ 1. 2. ❖ 1. 2. 3. ❖ o

Extension of inflammation from neighbor structure Pathology: Marked adhesions between parietal pericardium & surrounding structures as chest wall & diaphragm No hemodynamic or clinical significance Clinical picture: Clinical picture of associated valvular lesions Fixed apex: apex does not change its site with change of position of patient. Broadbent's sign : systolic retraction of lower sternum & posterior intercostal spaces with every heart beat Investigations: Kinking of barium filled esophagus synchronous with cardiac contraction

❖ Treatment:

o Of associated valvular lesion & Pericardiolysis(removal of adhesions) in severe cases..

38

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Diseases Of Myocardium

Myocarditis

Definition: Acute inflammation of the myocardium.

I ❖ Etiology 1. Infection: THE MOST COMMON CAUSE:

• Viral; commonest cause e.g. coxsackie virus ,

2. Idiopathic

adenovirus, CMV,HIV

3. latrogenic e.g. methyl dopa, penicillin,

• Bacterial e.g. streptocoeeal, diphtheria

sulphonamides, anti-tuberculous

• Parasites e.g. Trypanosoma eruzi, toxoplasma gondii • Spirochetal e.g. Lyme disease, leptospirosis.

4. Irradiation

5. Immunological: SLE,scleroderma

• Fungal «& Rickettsial Inflammation of heart :le

Clinical picture

»

I. Symptoms : Asymptomatic

• Palpitation, Chest pain. Dyspnea

• Diffuse myocardiai invoivement —> fulminant congestive HE -> biventricular failure II. Signs : Heart sounds: soft heart sounds, prominent S3, tachycardia Investigations

1. Chest X-ray : mild eardiomegaly

_

2. ECG: ST, T wave abnormality , arrhythmia, heart block especially in diphtheria, Lyme disease 3. Cardiac enzymes: mild increase

4. Endomyocardial biopsy: invasive, may show viral RNA by PCR. 5. Viral Ab titre : increased.

❖ Treatment 1. Treatment of tne cause.

2. General measures : bed rest

3. Symptomatic treatment: antibiotics, antiviral, antifailure & antiarrhythmic. 4. Specific treatment: Anti-inflammatory drugs: o

Steroids:controversial - limited value

o IVIG; IV immunoglobulin : high dose is effective. Cardiomyopathy V Definition

Primary myocardiai disorder that is not due to structural abnormalities of heart(valvular or congenital), ischemie heart disease & hypertension (systemic or pulmonary). Classification

i.

1. 2. 3. 4. 5.

Ciinical classification:

ii.

Restrictive cardiomyopathy Dilated cardiomyopathy Hypertrophic cardiomyopathy Arrhythmogenic right ventricular dysplasia. Unclassified Cardiomyopathy e.g. Takotsubo cardiomyopathy

Etiological classification:

1. Primary cardiomyopathy of unknown cause

2. Secondary cardiomyopathy with defined underlying cause

39

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Restrictive Cardiomyopathy

Restrictive

Diastolic dysfunction RHF TfteuepM valv*

Muscle layers are stiff arrd resist stretctiing for filling

ti

Dilated (or congestive)

Ddated Cardiomyopathy

Systolic dysfunction Biventricular HF

Chambers greatly enlarged Ventricle wails are thinner

Hypertrophic Cardiomyopathy

Puimortary Cinsulatlon

Hypertrophic

Diastolic dysfunction LHF

Smaller fUling areas

Ventricle walls greatly thickened

o Apical hypertrophy

0 Symmetrical(concentric)

0 Asymmetrical septal hypertrophy

hypertrophy

1^

MNMwOMCt namrnfttmn

\J|/— ~

■wwwiAaHi

m

riyyeilroRhy

o

Asymmetrical septal hypertrophy with obstruction : LV > RV

o

AS, PS, IHSS murmur.

1 Pulsus Bisferiens

[ i Murmur ]

t Murmur

bypertrophy MR

SAM

LVOT

gradient ASH

EARLY SYSTOLE

MITRAL LEAFLET. SEPTAL CONTACT

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Restrictive Cardiomyopathy (Non Dilated Non Hypcrtrophicd) I.

Primary:

• Idiopathic • Endomyocardial fibrosis • Eosinophilic endomyocardial disease (Loeffler endocarditis) II. o

Irradiation.

o Immunological: Scleroderma o

I. Primary: o Idiopathic o Familial: Hypertrophic obstructive cardiomyopathy (HOCM)= Idiopathic hypertrophic Subaortic stenosis (IHSS): autosomal dominant

infection 2.

Secondary:

Hypertrophic Cardiomyopathy

Dilated (Congcsti\ c) Cardiomyopathy ❖ Etiology : I. Primary: o Idiopathic, Familial. II. Secondary: I. Infection : myocarditis post

3.

latrogenic: alcohol, cocaine, cyclophosphamide Immunological: SLE,

Infiltrative: Sarcoidosis,

Scleroderma

amyloidosis, hemochromatosis. glycogen storage disease.

Infiltrative: Sarcoidosis,

pattern with mutations in beta myosin on chromosome 14

amyloidosis, hemochromatosis. Peripartum cardiomyopathy. Endocrinai: acromegaly, pheochromocytoma, thyrotoxicosis, myxedema,DM. Neuromuscular: Friedriech's

ataxia,, Duchence myopathy ❖ Pathogenesis & Hemodynamics

Fibrosis or myocardial infiltration —»t myocardial stiffness —> rigid inelastic muscle —> I ventricular compliance —»J, relaxation & J, diastolic ventricular (usually RV)fdling DIASTOLIC dysfunction ^ J. COP & SVC. No dilatation or hypertrophy except very late.

Heart

failure

dilatation

of

associate

with

one

both

or

ventricles.

II. Secondary: • Pheochromocytoma

Pathogenesis & Hemodynamics:

❖ According to the site of cardiac hypertrophy : I. Non-obstructive type: 1. Apical hypertrophy at the apex. 2. Symmetrical (concentric) hypertrophy: Hypertrophy involves the entire LV leading to diastolic dysfunction. 3. Asymmetrical septal hypertrophy

Decrease SYSTOLIC function

(general hypocontractility) All chamber are dilated + mural thrombi

without obstruction.

II.

Obstructive type: Hypertrophic obstructive cardiomyopathy(HOCM)= Idiopathic hypertrophic Subaortic stenosis (IHSS): • Asymmetrical hypertrophy (eccentric) of interventricular septum(LV > RV)—» causing obstruction of outflow tract of left

ventricle ^ leading to : I.

Encroachment on the aortic

opening —♦ picture of subvalvular AS i.e. left ventricular outflow

tract obstruction (LVOO)—> LVH ->LVF ^ low COP &

pulmonary congestion 2.

LVH^ DIASTOLIC

dysfunction (J, compliance) + pulmonary congestion 3.

Blood acceleration in the LV

outflow tract creating Venturi effect —> systolic anterior wall motion (SAM) of the anterior mitral leaflet—> MR and increases the outflow obstruction of the LV.

The septum may bulge into the outflow tract of the RV impending the ejection of blood from RV to pulmonary artery —> picture of subvalvular PS (giant a wave).

41

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

1. Symptoms & signs of RHF:low CO & systemic congestion 2. Symptoms & signs as constrictive pericarditis.

❖ Clinical picture 1. Pulse : 1) Manifestation of biventricular HF.

(usually refraetory CHF),LVF usually dominates

A. Jerky carotid pulse because of rapid ejection and sudden obstruction to LVOF during systole

B. Pulsus Bisferiens: pulse with two

Secondary MR & TR systolic peaks: Symptoms & signs of o The first is caused by rapid early LV ejection of blood into aorta. Pulmonary & systemic congestion o The second is caused by the backflow of the regurgitated blood of mitral valve by Venturi effect.

C. Later : weak pulse in LVF > N.B. Sudden arrhythmic death commonly in young adults during physical exertion. 2. Giant a wave.

3. Signs of LVH with double apical pulsation (powerful atrial contraction). 4. Symptoms & signs of LHF: low COP & PVC. 5. Auscultation :

a) AS murmur. b) PS murmur (infundibular). c) MR in 50% of cases due to distorted papillary muscle funetion d) Murmur of IHSS; late ejection systolic murmur due to LVOO during systole: o

.1. by squatting or leg raising (both T VR which fill ventricle & so .1 obstruction)

o

Murmur t with standing

e) 4"* Heart sound due to powerful atrial contraction

Investigations 1. Chest X-Ray:

o LV hypertrophy o Cardiomegaly o Pulmonary congestion (biventricular) o Pulmonary congestion thickening 2. ECG:low voltage, non-specific depressed ST segment & inverted T wave, arrhythmia & LV enlargement RV enlargement( mild & very late) LV & RV enlargement 3. Echocardiography & Doppler o Asymmetric hypertrophy of FV septum o LV & RV dilatation o Decrease ventricular cavity & o Mild & very late RV enlargement o No pericardial calcification o CT & MRI ;exclude pericardial

Decrease motion

o

o Very late dilatation

Generalized decrease

(septal wall thicket thickness exceed free

in wall motion, MR

wall thickness)

&TR.

Pedigree analysis reveals autosomal

4. Endomyocardial biopsy: shows the pathology

dominant inherited pattern. Treatment:

1. Medical: Treatment of HF & Treatment of complications e.g. Antiarrhythmics for arrhythmia e.g. Amiodarone & Anticoagulants for embolism + 1) Medical:

1. Medical :

■ Steroids in eosinophilic disease 2. Surgical:

■

1. Medical: Treatment of HF but avoid

vasodilators & positive inotropic (digitalis) they will f contractility -♦ "f

Steroids or

o

Resection of thick endocardium

immunosuppressives for myocardial

o

Heart lung transplantation

inflammation

outflow tract obstruction

o

Negative inotropic : BB, CCB J, obstruction & j, angina

2. Surgical: myectomy of hypertrophied septum. 42

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

N.B.:

iini I ni'tiriiiii

Cardiac tamponade Collstricti^ e pericarditis Clinically

Restrictive cardiomyopathv

o o

Pulsus paradoxus Prominent y descent

+

db

-

-

+

-

o

Prominent x descent

+

+

o Kussmaul's sign o S3 or pericardial knock

+

+

-

-

+

±

-

+

+

+

+

-

+

±

-

+

-

-

+

-

-

-

o

S4

-

-

ECG o

Low voltage

o

Abnormal P wave

o

Electrical alternans

+

o

Cardiomegaly

+

o

Pericardial calcification

o

Pericardiai effusion

1

Chest X-Rays -

I

Echocardiography o Pericardial thickening o Thickened myocardium

+

I

-

+

-

-

-

+

1

CT-MRI o Pericardial thickening o

Pericardial calcification

o o

Endmyocardial biopsy Expioratory thoracotomy

1

1

-

+

-

+

-

-

-

+

+

+

+

43

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Rheumatic Fever Definition

• It is an infiammatory disease due to auto-immune reaction that occurs as a sequel to upper respiratory tract infection with group"A" p hemolytic streptococci.

Etiology: Autoimmune theory,two mechanisms: 1.

Altered antigenicity:

• Binding of streptococcal antigens to tissue proteins alter their structure and II.

induce antibody formation against them. Cross antigenicity (antigenic similarity):

• Antibodies formed against streptococci react with human tissue antigens, because they ar^ immunologicaliy identical (cardiac myosin, and sarcolemmal membrane protein) ❖ Predisposing factors: /. Recurrent streptococcal infection: most importantfactor. 2. Age:

• Commonest age occurs between 5-15 years • Rare below 5 y. & after 25 y. 3. Sex:

•

Male as females

•

Chorea is commoner in females

4. Family tendency:

• Hereditary predisposition & similar environmental condition (overcrowding) 5. Country: developing country due to overcrowding. 6. Climate:

• Cold months —> 2 peaks : April & September ❖ Pathologv: CoHagcnIiben ribrioold collagen n«n»i(

Types B. Proliferative reaction

A. Exudative reaction

I*

*V'

Mural endocarditis

(MacCallum's patch)

lUMi

- s'-y * ' '/f--''

000000000000 AKhoffcrd HasnMcelH

1. Meninges

1. Heart:

2. Serous membranes : pleura, pericardium, a) Endocardium: MacCallum patch : o Thickened & roughened area in left atrium above the posterior peritoneum leaflet of mitral valve 3. Synovial membranes b) Myocardium: Aschoff bodies 2. Ligaments, periosteum, fascia 3. SC tissue 2. Form

o Fibrinoid degeneration, edema, inflammatory cells

• The main lesion is Aschoff nodules which consists of:

o Central fibrinoid degeneration

o Macrophages, lymphocytes, plasma cells and Aschoffs giant cells

o By resolution

o

Outer layer of fibroblasts

o

3. Healing Concentric fibrosis & scarring

44

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Clinical picture of Rheumatic Fever

Arthritis

Paucarditis

Disproportiouate tachycardia Tic Tac rhythm

Arrhythmias, heart block. ;i

Dry pericarditis [

MS:cusp edema j Chorea

SC nodules

Erythema margiuatum

%®=(X) 45

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

I.

Major criteria: Arthritis, Pancarditis, Chorea, Dermatological lesions(SC nodules & Erythema marginatum)

1) Arthritis (polyarthritis): 75 % of patients: o

More common in Adults.

o o o 0 0

Polyarticular affecting big joints (knee, ankle, elbow, wrist) Character: lesion may be Additive (overlapping) or migratory (fleeting) leaving the affected Joints free . Course: Each joint is affected for about a week, with total course of arthritis is 6 weeks. Dramatically response to Aspirin Examination: all signs of inflammation : hot, red, tender, painful, swollen, with effusion with limitation of movement

o Fate: Leaving joint with complete resolution 2) Pancarditis: 50 %: More common in children: A. Pericarditis : B. Myocarditis: o Dry or with effusion. a) SAN: 0 Very rarely adhesive o Tachycardia: disproportionate pericarditis tachycardia out of proportion o Never constrictive to fever, persistent with sleep. pericarditis. b) AVN: o Arrhythmias, heart block. c) Ventricles : •

Ventricular dilatation —> HE

■

Functional MR,TR.

C. Endocarditis (valvulitis):

❖ Any valve can be affected but the most common is MR, MS,AR, AS 1. Mild inflammation: resolution. 2. Moderate inflammation: MS:

a. Functional MS: Carey Coomb's murmur: mitral cusps edema —> narrowing of mitral orifice b. Organic MS: later on(2 years) due to

with diastolic gallop

valve fibrosis. 3. In severe inflammation: valve

• Tic-tac rhythm: weak SI due

rupture.

to loss of its muscular

30%:

component, so both SI & S2 are similar & equidistant due to tachycardia. 4) Subcutaneous nodules: 5) Erythema marginatum : o Painless non tender small o Painless non tender small erythematous swelling spots

Common in females

o

3) Rheumatic Chorea = Sydenham's Chorea, 10o

o Rapid involuntary jerky pseudo-purposive movements with hypotonia & emotional lability o Never with arthritis(> 6 months to manifest) II.

Minor criteria :

1. 2. o o o

Arthralgia Acute phase reactant: TESR t C reactive protein t Polymorphonuclear leukocytes 3. Past history of previous

Not adherent to skin but

adherent to deep structure, o On bony prominence and extensor surfaces of limbs, around joints & ligaments.

o

Starts as red macules which fade at the

center but remains red at the margin o Serpiginous margin, non-scarring o On Trunk & proximal extremities in crops.

III.

Other manifestations:

1. Bleeding per nose

2. Pallor, sweating, weight loss 3. Pleuris>y. Pneumonia 4. Peritoilitis

5. Eryth ema

nodosum.

JM-litM"" « Bp

rheumatic fever

IV. Complications of rheumatic fever: 1) Acute: arrhythmia & heart block, HP. 2) Chronic : a) Chronic valve lesions, rheumatic activity, adhesive pericarditis b) Jaccoud arthropathy:

• Rare late complications of rheumatic arthritis, where hands are deformed as with rheumatoid arthritis but ulnar

4. Pyrexia 5. Prolonged P-R interval

deviation of fingers can be voluntary corrected.

• This is due to lax CT surrounding joints of fingers resulting from recurrent joint effusion associated with recurrent

♦> Criteria for diagnosis: Revised (modified) Jones criteria for diagnosis of Rheumatic fever: A + B

A. 2 Major criteria or 1 major and 2 minors B. Evidence of previous streptococcal infection: 1 or more of: o Raised ASO titre > 250 units(or other strept. antibodies) o

episodes of rheumatic fever. Differential diagnosis: 1) Infective endocarditis 2) Causes of fever in cardiac patients 3) Other causes of arthritis, carditis, chorea

Positive throat culture 46

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

f

Ailblrcpli>l>iiin O titcr(ASO)

l>^ I +

I. Laboratory investigations: 1. Acute phase reactants: f ESR, +ve CRP,CBC (anemia - leukocytosis) 2. Antistreptolysin O tltre(ASOT): o Normally up to 150 Todd units o t Titre > 200 Todd units in adults or Titre > 300 in children indicates recent streptococcal infection o Rising titre is more significant 3. Anti-streptozyme test(AST): • Detects antibodies against five antigens of group A streptococci. • Elevation of one antibody is positive .

+

w.

m

m il . CSl

to kfln>l>iU

iiillillillllllwl

Rising titre is more significant

• False positive test is common. 4. Antifibrionlysin test. 5. Throat culture.

II. Cardiac investigations : 1) EGG:cardiac enlargement & arrhythmias 2) Chest X-ray : cardiac enlargement, pulmonary congestion in EVP 3) Echocardiography: cardiac enlargement, valve lesions.

I.

Prophvlactic

A. Prevention of occurrence : B. o o

Rapid treatment of any streptococcal sore throat + tonsillectomy for chronic tonsillitis. Prevention of recurrence : in patient with previous rheumatic fever, by prevention of streptococcal pharyngitis : Benzathine penicillin G(long acting):1.2 million units IM every month In penicillin sensitive patients : erythromycin orally 250 mg /12 hour Duration :

1) RF with no carditis:5 years after the last attack or until 25 years old whichever longer 2) RF with carditis but with no valvular heart disease: 10 years after the last attack or until 25 years old whichever

longer

3) RF with carditis & valvular heart disease: 10 years after the last attack or until 45 years old or for life. II.

Therapeutic:

A. General:

Bed rest till symptoms and signs of inflammation subside Diet: light meals, poor in salts. Symptomatic treatment: Rheumatic chorea : phenobarbitone, chlorpromazine

Heart failure:bed rest, anti-failure measures (diuretics - dilators - digitalis) C. Specific treatment: Drugs: APC A. Aspirin - Acetyl salicylic

B. Penicillin

C. Corticosteroids:

acid

❖ Action

o Anti-prostaglandins for

0 Acetyl salicylic acid :

o Antibiotics to eradicate p hemolytic streptococci ❖ Preparations & dosage: o Benzathine penicillin G:

-

■

Arthritis

60 mg / kg /day in 6 divided dose for 6 weeks

o Anti-inflammatory for Carditis

o Prednisone: 1 mg/ kg /day in 4

1.2 million Unit I.M. / week for 3

divided dose, for 4 weeks, with

weeks

gradual withdrawal by 2.5 mg / day

■

50,000 unit/kg/IM for children < 10 years old. o In penicillin sensitive patients : Erythromycin 250 mg /6 hour for 10 days

over next 4 weeks,

o

If no response after 2 davs.

/nethylprednisolone succinate 50

mg/kg/day IV on 3 successive days o Aspirin should be given during steroids withdrawal for additional 4

weeks to prevent rebound manifestations

47

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Endocarditis

*> Definition : Mi'iiiiViiriiiiiri i

- I 1 Ilnnffllttrmi^

• Inflammation of the endocardia! surface of the heart which may include heart valves, mural endocardium & septal defects.

1. Infective Endocarditis(lEC)classified as: • Acute , subacute, chronic or bacterial and non-bacterial. A. Acute bacterial endocarditis(ABE) B. Subacute bacterial endocarditis(SBE) o

Affects normal hearts

o

o High virulent organism (staph) in septicemia, or in addicts(Rt. sided

11.

Non-Infective Endocarditis

•

Affects diseased hearts

Rheumatic fever

• Systemic lupus erythematosus

o Caused by organism of low virulence

valves)

Etiology & predisposing factors:

I.

Underlying cardiac lesion:valvular lesions & septal defects:

❖ Essential for IE: high velocity jet stream: • Blood passing through a narrow orifice + high pressure gradient 1. Rheumatic heart disease: ❖ Common in : •

endothelial damage.

❖ Rare in:

• Right sided valves: tricuspid & pulmonary valves. • Common in regurgitation > stenosis e.g. MR > MS

Left sided valves: Mitral and aortic valves.

2. Congenital heart disease: ❖ Rare in low pressure gradient: • ASD,F4 • AF,congestive heart failure • Common in small narrow orifice > big wide orifice e.g. small VSD > big VSD ❖ Common in high pressure gradient: • VSD,PDA

3. Prosthetic valves: 20% of cases. 4. Pacemaker endocarditis

II.

Causative organisms & route of infections :

I) Gm+vecocci: •

Causative

o Streptococcus viridans

organism

(The most common)

%

• PDF,route of infection

o Dental(Tooth extraction) & oropharyngeal procedure (tonsillectomy)

o Streptococcus fecalis

o Staphylococcal Aureus

$ Ik

o GIT & Genitourinary procedures

o

Cardiac surgery & Catheterization

2) Gm-ve bacilli: o HACEK group : Hemopilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella

3) Others:Fungal or Rickettsial, Chlamydia 48

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

^ ❖ Pathogenesis:

o Endothelial damage occurs in top of damaged valves(abnormal surface) or due to high pressure jet of blood (abnormal flow).

o Sterile vegetations are formed from platelets and fibrin on the injured endothelium. o Infection ofthe vegetations is most likely when bacteremfa occurs with bacteria that adheres well to fibrin and platelets.

o Bacteria of low virulence can attach only to deformed valves and have slow rate of growth (subacute endocarditis), while virulent bacteria can attach to normal valve and may grow rapidly with severe toxemia(acute endocarditis). o

Manifestations of infective endocarditis mav result from:

> la; a) Vegetations may cause damage to the valves or occlusion of their orifices. o Infection may extend to the myocardium producing abscesses, conduction abnormalities and rupture of cordea, papillary muscles and ventricular septum b) Embolization of the detached vegetations with vascular occlusion. c) Immunoiogical reaction: formation ofimmune complexes that may precipitate in the tissues e.g. glomerulonephritis. d) Toxemia

49

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Clinical picture of Infective Endocarditis General

CNS

Mycotrc aneurysm

•

«35f

Pale toxic clubbing

Ocular

Acute AR,MR —> Sea gull murmur —> acute LHF & APO Roth spots Lung,

Abdomen,

Spleen

Renal

.-.ipr

' ,

MVO

Septic pulmonary embolism

Flea-bitten kidney

• Tender splenomegaly

• Stitching pain & splenic rub: splenic

Renal infarction Acute diffuse G.N.

infarction & perisplenitis

Muco-cutaneous lesions

d Splinter hemorrhage

Janeway's lesions

Osier's nodules

50

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Bad general condition Pyrexia of low grade & remittent Pale toxic look Pale toxic clubbing. Pulse : absent pulsations due to Ischemia and gangrene in UL & LL (arterial embolization)

1. Meningitis or Encephalitis. 2. Septic Encephalopathy, brain

■ ■

abscess.

In conjunctiva: petechial hemorrhage In retina : Roth spots by ophthalmoscope (oval hemorrhage & pale center)

3. Hemiplegia "Stroke". 4. Subarachnoid Hemorrhage due to ■ Sudden blindness due to rupture of mycotic aneurysm embolism of CRA occlusion (the arterial wall that have been weakened by infection in the vasa vasorum or where septic emboli have lodged ^ fibrosis ^ aneurysm —> rupture —> SAH) 5. Lung, Abdomen,Spleen 6. Renal affection 1. Flea-bitten kidney: 1. Lung: • Multiple small embolus causing • Septic pulmonary embolism microscopic hematuria may lead to lung infarction, lung

4. CVS

A. Signs of predisposing lesion. B. Murmurs:

1. Changes in the previously present(due to vegetation) 2. Development of new one due to: o

3. Ocular

2. CNS

I. General

1) 2) 3) 4) 5)

abscess or recurrent pneumonia. 2. Mesenteric vascular occlusion

with abdominal pain and GIT bleeding. 3. Spleen. • Tender splenomegaly • Stitching pain & splenic rub: due to embolization causing splenic infarction & perisplenitis

Perforated aortic or mitral

cusps —> acute severe AR or MR.

o Rupture of cordea tendinae —> acute severe MR.

Both leading to loud musical murmur(Sea gull murmur) —> acute LHF & APO. C. Heart Failure :

2. Renal infarction:

• Single large embolus causing gross hematuria 3. Acute diffuse G.N.:

• Autoimmune response to streptococci e.g. Nephritic syndrome, Nephrotic syndrome & GRP.

• Aggravation of valvular damage, toxic myocarditis 7. Muco-cutaneous manifestations:

A. Splinter hemorrhage: • Linear longitudinal bleeding under nail.

B. Janeway's lesions

• Small painless not tender •

Red

• Maculo-papular • In palm & soles • Due to rupture capillaries in groups.

❖ Complications 1. Toxic: infection & septic shock 2. Cardiac; valvular damage, HF 3. Embolic: renal failure, subarachnoid hemorrhage

❖ Differential diagnosis: •

Rheumatic fever

•

Causes of fever in cardiac

patients

• Other causes tender splenomegaly •

Other causes of GN & embolization

C. Osier's nodules

• Small painful tender •

Red

•

Nodules

• Pulp of fingers & toes • Due to immune hyperplasia of capillary endothelium ❖ N.B.: endocarditis of

IV drug abusers: 'Q* o SEE in general is more common on the left side, in the IV drug addicts the valves in the right

side are usually affected, e.g. tricuspid valve TR o Staph epidermidis is common in IV drug addicts.

❖ Modified Duke Criteria For Diagnosis of IE: o 2 major or 0 1 major + 3 minor or o

5 minor

A. Major criteria:

B. Minor criteria

1. Positive blood culture for typical organism of IE obtained from 2 separate culture e.g. Streptococcus Viridans

•

Positive blood culture not

meeting major criteria • Pyrexia > 38°C • Predisposing factor: heart disease or IV drug use

2. Positive echocardiography : Vegetation 3. New Valve regurge: o Development of new murmurs o Change of characters of already present one

• Immunological phenomenon e.g. GN,Osier's nodules

• Vascular phenomenon e.g. arterial embolism 51

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

i *♦*

I.

Investigations

Laboratory investigations : 1.

Blood culture: THE MOST IMPORTANT INVESTIGATION:

•

At least 3 samples should be taken during fever observed from 3 days up to 3 weeks.

•

Culture both aerobic & anaerobic.

•

Antibiotic sensitivity test:

to determine which antibiotic will be successful in treating the infection. •

Culture can be negative in:

o

Prior use of antibiotics

o 2. 3. 4.

bifection with other organisms e.g. HACEK, Fungi, Rickettsia Acute phase reactants : f ESR, +ve CRP, CBC (anemia - leukocytosis) Urine analysis : microscopic or gross bematuria, proteinuria Immunologic test: rheumatoid factor may be positive. Cardiac investigations : ECG & Chest X-ray : help in diagnosis of underlying cardiac diseases Echocardiography: Trans-thoracic Echocardiography: Visualize vegetations > 5 mm ( small vegetations can be missed) Detect underlying cardiac disease Transesophageal £cho or' Detect complications of SBE e.g. AR or MR TRANSESOPHAGEAL ECHOCARDIOGRAPHY is the best: detect small vegetations.

II.

1) 2) A. o

o o B.

[

Treatment: llii^biii I

Prophylactic antibiotic:

i.

Prophylaxis for dental procedures : only in high risk patients:

Patient with prosthetic valve 2. Previous episode of SBE 1.

3.

o

Allergy

Single dose 30-60 before the procedure

Antibiotics

children

Adult

o 0

Not allergic to penicillin o Amoxicillin or ampicillin o Clindamycin Allergic to penicillin : ii. Prophylaxis for non-dental procedures:

o o

2 gm orally or IV 600 mg orally or IV

o o

50 mg/kg orally or IV 20 mg/kg orally or IV

Antibiotic prophylaxis is not recommended, only needed in high risk patients, in invasive procedures are performed in the context of infection

Therapeutic :

II.

A. General:

1. Bed rest till symptoms and signs of inflammation subside. 2. Diet: light meals, poor in salts.

B. Symptomatic treatment: Heart failure : bed rest, antifailure measures (diuretics - dilators - digitalis) C. Specific treatment; Antibiotics :

Once suspected SBE, blood samples are taken for culture & treatment is started immediately with bactericidal drug singly or in combination till result of culture occurs, and change accordingly. 2. For staphylococcai & gram negative bacilli: Penicillin G 20-30 million units IV daily for 4 weeks plus • Cloxacillin 2 gm /4 hours IV for 4 weeks plus Gentamycin 1 mg / kg 8 hourly IV for 2 weeks Gentamycin 1 mg / kg 8 hourly IV for 2 weeks

1. For streptococci: •

o If the patient is allergic to penicillin or in patients with prosthetic valves, penicillin is replaced by Vancomycin 15mg/kg/12 hr IV.

3. Fungal endocarditis : amphotericin B D. Surgical treatment:

o Replacement of damaged valve e.g. valve rupture

o Replacement of valve in in resistant cases e.g. infected prosthesis. 52

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Subacutc Bacterial Endocarditis(SBE)

Rheumatic Fever

❖ Definition • It is an inflammatory disease due to auto-immune reaction that occurs as a sequel to upper respiratory tract infection with group "A" p hemolytic streptococci.

•

| 1

Affects diseased heart valves

• Caused by organisms oflow virulence

♦> Etiology & predisposing factors: ❖ Autoimmune theory: Altered antigenicity & antigenic similarity ♦> PDF: Recurrent streptococcal infection. Age, Sex, Family tendency. Country, Climate.

defects} & Infection. ❖ PDF: route of infection

❖ Pathology:

•

Exudative reaction

•

Proliferative reaction

1

❖ Underlying cardiac lesion {valvular lesions & septal

|

• Vegetations : organisms + fibrin + platelets • Toxic, Immune, local cardiac lesion & Murmur, Embolic ❖ Clinical picture:

Major criteria: Arthritis, Pancarditis, Chorea, Subcutaneous

General

Minor criteria :

Bad general condition , Pyrexia of low grade & remittent , Pale toxic look. Pale toxic clubbing, Pulse : absent pulsations.

Arthralgia

CNS

nodules. Erythema marginatum

Acute phase reactant;t ESR -1 C reactive protein

Septic Encephalopathy, brain abscess. Meningitis or

leucocytosis

Encephalitis.

Past history of previous rheumatic fever

Hemiplegia (Stroke), Subarachnoid Hemorrhage .

Pyrexia

Ocular: Petechial hemorrhage. Roth spots, sudden

Prolonged P-R interval

blindness

Others:

CVS:

Bleeding per nose Pallor, sweating, weight loss Pleurisy, Pneumonia,

Signs of predisposing lesion. Murmurs:

Changes in the previously present. Development of new one due to: Perforated aortic or mitral cusps acute severe AR or

Peritonitis

Erythema nodosum Complications: Acute : arrhythmia & heart block, heart failure Chronic : chronic valve lesions, rheumatic activity, adhesive pericarditis, Jacoud arthropathy DifTerentia! diagnosis:

MR

Rupture of cordea tendinae —» acute severe MR both leading to loud musical murmur "Sea gull murmur"

acute LHF & APO.

Heart Failure.

Infective endocarditis

Lung: Septic pulmonary embolism may lead to lung infarction, lung abscess or recurrent pneumonia Mesenteric vascular occlusion with abdominal pain and GIT bleeding. Spleen: Tender splenomegaly. Splenic infarction Renal: Flea-bitten kidney. Renal infarction. Acute

Causes of fever in cardiac patients Other causes of arthritis, carditis, chorea

diffuse G.N.

Muco-cutaneous manifestations: Splinter hemorrhage, Janeway's lesions. Osier's nodules N.B.: IV drug abusers: right side—> TR,Staph epidermidis is common in IV drug addicts. 10. Complications: Toxic : infection & septic shock

Local cardiac: valvular damage, HP Embolic: renal failure, subarachnoid hemorrhage 11. Differential diagnosis: Rheumatic fever,

Causes of fever in cardiac patients Other causes tender splenomegaly, GN & embolization. 53

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Criteria for diagnosis

❖ Revised (modified) Jones criteria for diagnosis of Rheumatic fever: A + B

A. 2 Major criteria or 1 major and 2 minors B. Evidence of previous streptococcal infection : 1

❖ Modified Duke Criteria For Diagnosis of IE : 2 major or 1 major + 3 minor or 5 minor: A. Major Criteria B. Minor Criteria 1. Positive blood culture for

1. Positive blood culture

typical organism of IE

not meeting major

or more of:

o Raised ASO litre > 250 units (or other strept. antibodies) o

Positive throat culture.

obtained from 2 separate culture e.g. Streptococcus

2. Pyrexia>38°C

Viridans

3. Predisposing factor:

2. Positive echocardiography: Vegetation 3. New Valve regurge : ■ Development of new murmurs

■

Change of characters of already present one

criteria

heart disease or IV

drug 4. Immunological phenomenon e.g. GN ,Osier's nodules

5. Vascular phenomenon e.g. arterial embolism

❖ Investigations: I.

11.

Lab.

Cardiac

® • • ®

Acute phase reactants. • Blood culture. Antistreptolysin O titre(ASOT) • Acute phase reactants Anti-streptozyme test(AST) • Urine analysis Antifihrionlysin test. • Immunologic test

e

Throat culture.

e

EGG.

«

e Chest X-ray. • Echocardiography.

ECG

• Chest X-ray. • Echocardiography. ❖ Treatment:

I.

A. Prevention of occurrence: tonsillectomy

Prophylactic:

o In high risk patients in dental procedures ■ Not allergic to penicillin: Amoxicillin or ampicillin ■ Allergic to penicillin: Clindamycin

B. Prevention of recurrence:

o Benzathine penicillin G o In penicillin sensitive patients ; erythromycin II.

Therapeutic:

1. General: bed rest & diet.

2. 3. o o

Symptomatic:antifailure treatment for heart failure (diuretics - dilators - digitalis) Specific treatment: Drugs Aspirin: for Arthritis 0 For strentococci: Penicillin G nlus Gentamvcin Penicillin for p hemolytic streptococci 0 For staphylococcal & gram negative bacilli :

o

Corticosteroids for Carditis

Cloxacillin nlus Gentamvcin

0 Fungal infection: amphotericin B 4. Surgical treatment: replacement of damaged valves

❖ N.B. Blood supply of the heart Anastomosis

Left coronary

(junction of vessels)

artery

Right coronary

Circumflex artery

artery

Marginal artery Posterior interventricuiar

Anterior interventricuiar

artery

artery 54

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Coronary Artery Disease(CAD) *> Blood supply of the heart by right & left coronary artery:

B. Left coronary artery • Left posterior aortic sinus

A. Right coronary artery

o

Origin

•

o

Site

• Runs in the atrioventricular groove

o

Branches

1. Marginal artery 2. Posterior (Inferior) interventricular branch

Anterior aortic sinus

• Runs in interventricular groove (anterior then inferior) 1. Circumflex (anastomose with right coronary) 2. Anterior interventricular branch

which anastomose with anterior interventricular

o Ends by o

branch of left coronary • Anastomosis with circumflex branch of left coronary I.

Pattern

Balanced circulation :

• Right coronary supplies RV and posterior part

of

• Lt coronary supplies LV & anterior part interventricular septum II. Left coronary predominance :

of interventricular septum (IVS) II. Right coronary predominance :

coronary

supply

• Right coronary supplies as well as the posterior part of LV.

• Left coronary supplies as well as posterior part of IVS & posterior wall ofRV

o

Coronary • Most of the veins drain in coronary sinus, which lies in the AV sulcus & drain in right atrium veins

❖ Coronary Artery Disease(CAD): Cerebral cortex-

Thaamus

Detinition:

Hypothalamus -

Imbalance between the supply of oxygen and the myocardial demands •

lechanism ofishbemic p

Medulla

Myocardial ischemia leads to local accumulation of metabolites (lactic acid, kinins, adenosine) which stimulate nerve endings.

C8-T4

Pain impulses are transmitted through cardiac sympathetic fibers to lower cervical and upper 4 thoracic spinal segments. Somatic nerves

Pain is felt in corresponding peripheral dermatomes.

from skin, muscle, bone of thorax, arms,dermatomes

Modes of presentation of coronary artery disease 1) Stable angina pectoris. 2) Acute coronary syndromes:

Stomach/lower

oesophagus

Unstable angina B. Non ST segment elevation MI C. ST segment elevation MI 3) Silent(painless) infarction: 5- 15 % of cases, causes: A. Most cases are unknown | : pain threshold B. Masking symptoms: severe HF,arrhythmia. C. Lost sensation:

1. Old i^e, Autonomic neuropathy(DM,Tabes dorsalis)

I j 2. Anesthesia, After cardiac transplantation [ 3. Shock, Syncope 4) Heart failure. 5) Arrhythmia & conduction defects. 6) Sudden death.

55

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Angina pectoris (.Myocardial Ischemia)

Myocardial infarction (Ml)

❖ Definition:

o Transient imbalance between myocardial demand and cardiac oxygen supply without myocardial necrosis.

o Permanent imbalance between myocardial demand and cardiac oxygen supply with 1 myocardial necrosis due to prolonged cessation of blood supply > 30-40 min. ❖ Etiology, predisposing factors:

I.

Decrease coronary blood flow :

Fibrofatty plaque

A. Blood factors:

Complicated plaques

1. I Qualitative : anemia, hypoxia e.g. F4 2. I Quantitative: low CO,low BP B. Vessel factors: 1. o

Coronary Atherosclerosis: the most common cause. Partial or total occlusion of coronary artery by coronary thrombosis on top of coronary atherosclerosi >.

2.

Blood disease e.g. DIC,PRY

3.

Coronary spasm : Prinzmetal angina

LIpids

L.

Core-

Foam cell

Thrombus

Smooth-muscle celM

Calcification-'

4. Aortic Dissection 5. Embolic : infective endocarditis

6.

Vasculitis : SLE(?),PAN((?)

Increase oxygen demands: I Myocardial contractility e.g. ventricular hypertrophy, AS. t Preload : HDC,fAfterload : HTN Predisposing factors: III II

A, Non modifiable:

Age > 45 years old Sex: male > female (4:1), Positive family history Personality: Type A

❖ Atherosclerosis:

B. Modifiable:

• • • •

❖ High risk: ❖ Low risk: • Obesity, f Saturatedfat in diet Hypertension Hyperglycemia(DM) • J, Physical activity • Psychological Stress, Polycythemia Hyperlipidemia • Hyperuricemia, Homocystinemia Cigarette smoking ❖ Pathogenesis : o Atherosclerosis with ruptured atheromatous

Definition:

It is a progressive inflammatory disorder ofthe arterial wall characterized by formation offocal lipid rich deposit of atheroma —> ischemia. Pathogenesis : sequence of events : Endothelial dysfunction —► protective response results in production of cellular adhesion molecules —♦ recruit inflammatory cells predominantly monocytes —> monocytes migrate into intima, differentiate into macrophages and ingest lipid to form foam cells —> cytokines and growth factors produced by activated macrophages induce smooth muscle cell migration into intima migrating smooth muscle cells change from contractile to synthetic phenotype —>■ the smooth muscle cell & macrophages accumulate LDL from the plasma, this is enhanced by f LDL in blood —»fatty streaks & plaque (atheroma) formation.

plaque with superimposed occlusive thrombus leading to complete cessation of coronary perfusion —> ischemic necrosis of a localized area of the myocardium. Thrombus

Thrombosis •ft ■

i

in

6hr Necrosis

1

Lesions of atherosclerosis : A.

B.

C.

The fatty streaks: Thin flat yellow streaks in the intima, they consist of macrophages and smooth muscles cells whose cytoplasm distended with lipids (foam cells). Fibrous atheromatous plaque

Stiff myocardium, S4 2 weeks

Soft granulation tissue (myomalacia cordis), S3

It consists of fibrous cap under the endothelium & lipid zone consists of lipid laden macrophages. Complicated piaque: Complicated plaque is calcified and fissured or ulcerated ^ rupture plaque —> non-occlusive thrombosis

2 months

Avascular non contractile scar

(healed - old infarction).

56

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

1

Exhaustion & fatigue.

Exertional Dyspnea due to myocardial fibrosis (J, LV compliance).

HicCough & eructation due to acute indigestion.

complications : within first few days

Modes of presentation; early

pain

Due to severe

o

CL/P; Hypotension & bradycardia

stimulation

o Leading to vagal

o

o

o

infarction 57

RV infarction due to extension of inferior

LVF

B. RVF due to;

A. Acute LVF due to extensive MI —> APO

8. Heart Failure:

from DVT.

1.

o

o

7.

o

o

motor weakness.

& corticosteroid

Responds to physiotherapy Complications of treatment; Complications of prolonged bed rest e.g. orthostatic pneumonia, DVT, osteoporosis Complications of drug therapy especially anticoagulants.

corticosteroid

o

ischemia, fibrosis Responds to

limitation of movement —>

myocardium .

Due to reflex spasm from pain + reflex sympathetic VC —> Auto-immune response due to release of protein from damaged

leukocytosis.

shoulder with edema, sweating of left hand with

Pain & stiffness of left

Pleuropericardits, with fever, chest pain, pericardial, pleural rub &

o

o

Post- MI syndrome Shoulder-hand syndrome Developing weeks after MI

o

6. Frozen shoulder syndrome

Ventricular REmodeling : Soon after MI, LV begins to dilate due to expansion of infarcts with hemodynamic impairment and appearance of chronic congestive heart failure occurring weeks or months after MI.

Ventricular REinfarction.

Examination: double apex, weak SI, diffuse pulsation. Echocardiography ; aneurysm + paradoxical pulsation ECG : persistent elevation of S-T segment for > 6 weeks

Diagnosis :

Recurrent arrhythmia Rupture with hemopericardium

Recurrent embolism

Refractory HF

Clinical picture & Complications :

Definition : dilatation of week scar in the wall of LV

Anginal attacks. Ventricular Aneurysm:

Late complications after several weeks

5. Dressler's syndrome

4.

3.

C.

B.

A.

o

D.

C.

B.

A.

o

o

2.

II.

Myocardial infarction

Systemic embolism: due to mural thrombi in LV Pulmonary embolism: either from right sided gnt siaea mural thrombi on top of infarcted septum or A. tum or

ThromboEmbolism :

RVF.

acute LVF —>■ acute pulmonary oedema (APO) Perforation of interventricular septum —> acute

E^A. Rupture of ventricular wall with fatal hemopericardium. B. Rupture of papillary muscle with acute MR

VT/VF Rupture mvocardium ;

ii.

Sudden Death:

& tachycardia

"t

infarction(> 40J % /O) } o Leading to pump failure CL/P; Hypotension

Due to massive

B. Neurogenic shock

Collapse(Shock); of2 types;

Dry with pericardial rub, Hemorrhagic effusion

Pericarditis:

Most serious —> ventricular tachycardia & HB

Arrhythmia ; (W\AAAAAA The commonest^ extrasystoles

Painless MI: see before.

A. Cardiogenic shock o

Symptoms:

Chest pain :acute chest pain as angina but; More severe & more prolonged > 20 min May occur without precipitating factor Not relieved by rest or nitroglycerin

1.

❖

6. i.

Heavy meals {postprandial angina due | to oxygen demand in splanchnic vascular bed) Relieving factors:rest- nitroglycerin. II. Atypical presentation: No chest pain. Angina equivalents, common in elderly: Angor animi(fear of death)from vagal stimulation; fainting, vomiting, dizziness. Palpitation due to arrhythmia.

Hypoglycemia.

Sexual intercourse

Emotional stress

(angina of effort)

Physical exertion: exertional angina^

Stress:

Cold weather

unstable angina. Precipitating factors:

Never < 30 sec or > 30 minutes except

1-5 minute up to 20 minutes

Duration:

Dull aching, constricting, compressing, strangling, suffocating. Never pricking, stitching or throbbing.

Character:

cardiac apex

Epigastrium, back to interscapular region Never infra-mammary or localized to

Neck & lower jaw.

, Start centrally & extends peripherally to: Shoulders, arm,forearms (especially left)

Radiation:

I. Typical presentation: typical chest pain: Site: diffuse retrosternal or precordial

.Viigina pectoris (.Myocardial Ischemia)

1 1

❖ Signs; Mvocardial infarction Angina pectoris (Myocardial Ischemia) o Clinical picture absent in small infarction & causes of o In between attacks; may be negative

painless MI

or signs of etiology.

*1* During attack o Pallor, sweating, restlessness, cold skin o Pallor, sweating, restlessness, cold Genera

o Fever: due to absorption of necrotic tissue, start from

skin

2nd day, last for I week.

I

o JVP: t in congestive HP & RV infarction ❖ Rate :

o Rate : tachycardia o Rhythm: arrhythmia & heart block

o

Normal: in mild cases

o Tachycardia: cardiogenic shock, HF, Anterior MI o Bradycardia: neurogenic shock, HB,Inferior MI ❖ Rhythm : arrhythmia & heart block Transient Hypertension : pain. Anterior MI (sympathetic overactivity) Decrease BP in shock, inferior MI (Parasympathetic overactivity)

2. Pulse :

o Transient Hypertension 3. BP:

Decapitated BP (if previously hypertensive patient, marked j in systole with slight J, in diastole) 4. Cardiac examination: due to myocardial injury: o

SI: Weak

o S2: Paradoxical splitting due to delayed closure of aortic valve S3 due to flahhy myocardium,in HF & shock

5.

S4: J. ventricular compliance by ischemia / infarction resulting in high ventricular pressure Pan-systolic murmur of MR: papillary muscle ischemia Pan-systolic murmur of VSD: rupture ofIVS Others o ± Signs of LVF or RVF ❖ 1. 2. 3.

❖ Grades of angina hy Canadian Cardiovascular Society: 1) Grade I: no limitation of physical activity with ordinary effort, angina occurs on prolonged strenuous physical activity.

Who diagnostic criteria: Typical chest pain (> 20 min.) Typical ECG changes Typical enzymes:f CK-MB - troponin

2) Grade II: slight limitation of ordinary activity, angina on climbing > 1 flight of stairs. 3) Grade III: marked limitation of ordinary activity, angina on climbing 1 flight of stairs. 4) Grade IV: severe limitation of

DD:

physical activity, angina at rest 1. Other causes of acute chest pain 2. Other causes of chest pain of cardiac origin 3. In Prinzmetal angina: other causes of

1. Moderate infarction: from other causes of acute chest

pain: see later 2. Massive MI:from other causes of acute chest pain,

acute dyspnea, acute RVF & shock: massive pulmonary embolism, massive lung collapse &

ST elevation

tension pneumothorax.

3. Other causes of ST segment elevation. DD of ST segment elevation : 2. Prinzmetal angina

1. Acute pericarditis ©

3. AMI®

P

1. Elevated ST

o Concave up in all leads

o

Flat in some leads

o Convex up(coved)in some leads

2. Pathological Q

o

Absent

o

Absent

o

Present

3. Cardiac enzymes

o

Normal

o

Normal

o

Elevated

58

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

plaque(Atheroma).

relieving factors) during the

)jr"l

intermediate syndrome

❖ ECG:

3. Angina at rest lasting > 10 minute.

treatment.

by less exertion, with resistance to

2. Crescendo angina: worsening of preexisting stable angina: more severe & frequent or precipitated

months

1. Angina of recent onset: < 2

on top of atheroma. ❖ Clinical picture

plaque (Atheroma)of the coronary arteries

Fixed stable atheromatous

angina Invers

(transmural ischemia)

o Complicated (fissured or ulcerated) o Normal coronary arteries or atheromatous plague narrowed lumen (atherosclerosis) o N.B. positive ergometrine test —> localized spasm

❖ Angiography shows:

o Transient flat S-T segment elevation during attack of pain

o Usually at young age.

not related to exertion,

o It is angina at rest not precipitated by increased myocardial oxygen demands i.e.

o Stomach in peptic ulcer or hiatus hernia & gall bladder in cholecystitis —> cholecystic heart,

7. Linked angina: pain is referred to nearby diseased organ e.g.

flPP

o Angina occurring at night and may wake up the patient from sleep. It may be provoked by vivid dreams.

digitalis. 6. Nocturnal angina:

o Angina occurring in Syphilitic AR due to coronary osteal stenosis characterized by being nocturnal, prolonged, associated with autonomic disturbance (sweating, tachycardia), not relived by nitroglycerin & may respond to

5. Angina of Lewis :

jCBmcal Types

3. Variant angina = prinzmetal angina = vasospastic angina =

4. Angina decubitus : in LSHF, precipitating by lying down & improves on sitting.

o

attack

o S-T segment depression occur in ECG with effort or during anginal

preceding 2 months

❖ Type:

2. Unstable Angina = intractable angina, pre-infarction angina,

J

❖ Etiology: It occurs when coronary perflision is impaired by: o Complicated (fissured or o Coronary vasospasm either in ulcerated) atheromatous plague i.e. normal coronary arteries or in non occlusive coronary thrombus top of atheromatous plaque.

Fixed stable atheromatous

o Classic type where pains did not change their character (severity, precipitating &

o

nil

= angina of effort

1. Stable Angina = classic angina = exertional angina

Angina pectoris(Myocardial Ischemia)

T-depresslor

jS. Diagnosed by cardiac enzymes.

segment Elevation MI

4. Associated with NSTEMI: Non ST

wall

third to one half of the ventricular

3. Infarction limited to the inner one

thrombosis

1. Previously called subendocardial MI 2. Occur without superimposed

B. Non Q-wave MI, NSTEMI:

ST depression

SubendocardiaJ injury;

5. Confirmed by cardiac enzymes.

Elevation MI

4. Associated with STEMI: ST segment

ventricular wall.

3. Infarction of full thickness of the

thrombosis

1. Previously called transmural ML 2. Occurs with superimposed

A. Q-wave MI,STEMI:

ST elevalion

Transmural (epicardlal) injury

❖ Myocardial infarction according to Q wave & ST segment:

❖ Investigations:

❖ Myocardial infarction

❖ Angina pectoris(Myocardial Ischemia)

NSTEMI STEMI

1. ECG:

ST segment Depression

Flat STE:

Prinzmetal angina

A. Resting ECG:

Q wave MI:

Hyperacute T wave: seconds to minutes

A. Between attacks may be normal.

after infarction, the earliest sign.

B. During attack :

Ischemic pattern : inverted T wave Injury pattern : raised convex ST

1. ST segment changes : o Depression. o Elevation (flat) in Prinzmetal angina

segment elevation —> recent infarction Infarction (Necrosis): Pathological Q. Late ,S-T & T return gradually normal,

2. T wave changes : o

Flat or inverted.

only remaining is pathological Q = Old

3. Arrhythmia & heart block

infarction B.

Non Q-wave Ml:

o

ST segment depression ± inverted T wave.

:

Indications:

Indications:

1. Stable or unstable angina refractory to treatment

1. Acute coronary syndrome with unstable hemodynamics or rhythm 2. Unstable angina and Non-ST elevation MI

2. Patients who are candidate for

3. ST elevation MI.

coronary revascularizatlon 3. Post infarction angina (Recurrent)

4. Unexplained significant chest

pain where the diagnosis of angina is uncertain. 5. For risk factors of Atherosclerosis :

Hyperlipidemia : Cholesterol, LDL,HDL & TG Hyperglycemia : glucose to exclude DM Hyperuricemia: uric acid & Homocystenemia: homocysteine Acute coronary syndromes(ACS): Chest pain

❖ N.B,:

ECG:

ST elevation MI

Non ST elevation MI

o Elevated Enzymes: Non ST elevation MI

Classification:

1. Primary Hyperlipidemia: Due to defect in genes and or enzymes involved in lipoprotein metabolism or transport ❖ Lipids elevated: ❖ Lipoprotein elevated: Types o Cholesterol o Triglycerides ■ Chylomicrons o Type I ■ T ■ T o Type Ha

■

LDL

o Type lib o Type HI

■

VLDL, LDL

■

IDL

■

Chylomicrons

o Type IV

■

VLDL

0 Type V

-

VLDL

■

Chylomicrons

■

T

■ t . 1 ■

Normal

. 1

■

Normal

"

T

■

T

■

T

■

t

65

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

2. Secondary Hyperlipidemia: ❖ Causes:

1. Alcohol excess,

2. Drugs : Non selective pB, Corticosteroids, Diuretics 3. DM,Nephrotic syndrome& Hypothyroidism. 4. Obstructive liver disease. Chronic renal failure.

Clinical picture & complications:

Of the cause Atherosclerosis

Pancreatitis (if TG > 500 mg/dL)

Xanthelasma :subcutaneous deposits of cholesterol just medial to the eyelids Plane xanthoma: on palmer creases Tuberous xanthoma: over the joints; elbow & knees.

Tendinous xanthoma: over tendons e.g. Achilles tendon ,patellar tendon & finger extensor tendons Eruptive xanthomas: occur on the buttocks, posterior thighs ♦> Desirable levels:

Treatment of dyslipidemia:

o Diet control, regular exercise & weight loss, o Drugs : lipid lowering agents

o

HDL

o

LDL

o

Triglycerides

o

Total Cholesterol

> 50 mg/dl j,TG synthesis ^ f catabolism of TG rich VLDL —> J, VLDL levels

Side effects:

o

o

e.g. Mechanism of

Myopathy & GB stones

GIT upsets

66

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Pulmonary Hypertension (P.HTN = PH)

Definition:

o

Normal pulmonary pressure is 25 /lO i.e. mean 15 mmHg.

o P.HTN is elevation of pulmonary artery pressure > 35/15 mm Hg i.e. mean > 20 mmHg Etiology: pulmonary hypertension

ONLT group 1 IS CALLED

HEABT

PULMONABT "ABTEBIAL" HTPEBTENSION.

CAT(hT

BUT ALL 5 GROUPS AWT BE REFERRED TO ELEVftTED mean ABTEHtAL

AS PULMONABT HTPEBTENSION QPH)

PBE59UBE > 25 MMH3 AT BEST ASSESSED BT BI&HT HEABT GROUP 3

CATHETEBIZATION

PH DUE TO LUNG DISEASE AND/OB CATE&OBIZED

HTPOXEMIA

INTO FIVE &BOUF>S BT THE WOBUD HEALTH

OE&ANIZATION CVWO group H-. GBOUP 1: PH DUE CHBONIC

PULMONABT ABTEBIAL THBOMBOEaWOLISM

HTPEBTENSION CPAH)

(meow?)

PAH EXAMPLES. GBOUP 2.

IDIOPATHIC, INHERITED,

PH DUE TO UFT

DRUG AND TOXIN INDUCED,

GBOUP 5; PH WITH UNCLEAR

HEABT DISEASE

CAUSED BT CONNECTIVE TISSUE

CMOST COMMON')

disease, HIV. SCHISTOSOMIASIS

MULTIFACTOEIAL mechanisms

WHO Group 1: Pulmonary arterial hypertension (PAH): 1. Idiopathic, Inherited , latrogenic : e.g. amphetamine & Infection e.g. HIV infection 2. Connective tissue disease & Congenital heart diseases 3. Bilharziasis & Portal hypertension B. Persistent pulmonary hypertension of the newborn C. Pulmonary veno-oeclusive disease & pulmonary capillary hemangiomatosis H. WHO Group 11 : Pulmonary hypertension secondary to left heart disease: 1. Left ventricular systolic dysfunction 2. Left ventricular diastolic dysfunction i.

A.

3. Left ventricular outflow obstruction

4. HI. 1. 2.

3. 4.

IV. 1. 2. 3.

Valvular heart disease e.g. MS & Pulmonary Venous stenosis

WHO Group 111 : Pulmonary hypertension due to lung disease & chronic hypoxia: Chronic obstructive pulmonary disease(COPD) Obstructive sleep apnea Interstitial lung disease Mixed obstructive & restrictive pulmonary diseases WHO Group IV : Chronic arterial obstruction Chronic thromboembolic pulmonary hypertension Pulmonary vasculitis e.g. SLE Parasitic infection (hydatidosis) WHO Group V : Pulmonary hypertension with unclear or multifactorial mechanisms

1.

Hematologic diseases: chronic hemolytic anemia e.g. Sickle cell disease

2.

Systemic diseases: Sarcoidosis

3.

Metabolic disorders: Glycogen storage disease & Gaucher disease

❖ Symptoms & signs of: o General : of the cause e.g. COPD o Cardiac: low COP,RYE ± RVF, RAE,SIGNS OF P.HTN. 67

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Signs Of Pulmonary Hypertension Expiration

Inspiration Pa

Narrow physiologic

splitting (tP2)

JL S2

Pulmonary Area (Z""* left):

LA Si

Inspection & Palpation

83

Percussion

TR,S4

Auscultation

A. General examination: neck| vein: JVP with giant a wave B. Cardiac examination:

1. Combined Inspection & Palpation:

• Pulmonary pulsation in 2"'' left intercostal space +++++

• 2. 3. • • • •

Diastolic shock: palpable accentuated pulmonary component of S2 Percussion: dullness in pulmonary area(pulmonary artery dilatation) Auscultation over the pulmonary area: t S2 Close splitting of S2 with accentuated pulmonary component Systolic ejection click Systolic ejection murmur due to functional pulmonary stenosis (due to dilated pulmonary trunk without dilated pulmonary ring) • Soft early diastolic murmur due to functional pulmonary regurge(Graham steel)(due to dilated pulmonary ring). 4. Auscultation Over tricuspid area: S4(t RV pressure) «& Functional TR(RV dilatation) 5. Signs of RVE&RVF. Complications:

1. RVE ± RVF (cor pulmonale) 2. Eisenmenger syndrome (shunt reversal) Investigations: 1. Chest-X-ray: o

Features of cause

o Aneurysmal dilatation of right and left pulmonary arteries as in primary pulmonary hypertension o Peripheral pulmonary oligemia, Right atrial & ventricular enlargement 2. CT, MRl, V/Q scan, pulmonary function test. Alveolus

3. 4. o o

ECG : R.A enlargement(P pulmonale), RVE,Right axis deviation, RBBB Echocardiography : Pulmonary artery dilatation, RVE,RAE TR: estimates pulmonary artery systolic pressure

o

Can detect cause as MS

Balloon

inflated' Pulmonary

capillary Puknonafy vein

Pttlmonaiy

artery

5. Right heart catheterization, pulmonary capillary wedge pressure : o The gold standard for diagnosis of PH . Righi

Treatment:

venlride

1. Treatment of the cause. 2.

ventncie

Oxygen: best treatment of hypoxic VC

3.

Anticoagulants : used for prophylaxis against secondary thromboembolism

4.

Reduction of Pulmonary Pressure :

Bosentan : endothelin receptor antagonist.

Phosphodiesterase 5 Inhibitors e.g. Sildenafil Prostanoids:

Iloprost: inhalation o Treprostinil: IV or SC o Epoprostenol: IV VD as CCB & ACEI.

Treatment of right-sided HF: best response is to Diuretics ± Digoxin. Heart lung transplantation is the only radical treatment. 68

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Pulmonary Embolism (PE) Etiology:

1. Deep venous thrombosis(DVT): the commonest(95%): DVT is promoted by 3 factors = Virchow's triad: A. Vascular trauma or inflammation causing endotheiial Injury.

B. Increased coagulability either inherited or acquired,(see later) C. Reduced blood flow (stasis): prolonged bed rest, HF, varicose veins

Detached blood c ot

2. Detached thrombi from right side of heart: A. Thrombosis in right atrium e.g. AF B. Vegetation of SBE Blood

C. Mural thrombosis in right ventricle e.g. RV infarction

clot

3. Embolism :

A. Amniotic fluid embolism, Air embolism & fat embolism. B. Paradoxical embolism : in left to right shunt(VSD, ASD)

❖ Clinical picture: I. II.

Of the cause e.g. DVT Depends on the size of emboli:

I. Small minute emboli:

o Asymptomatic o Recurrent showering of small minute emboli and cough.

PH

2. Moderate size embolus :Pulmonary infarction

o acute decrease in cerebral and coronary blood flow. 1) • • 2)

Acute chest pain as MI due to : I COP —i-J, coronary blood flow Reflex spasm of coronary arteries Acute dyspnea & cyanosis:

• Due to lung collapse • Local release of serotonin & thromboxane Ai from General:

platelets —> Pulmonary VC & bronchospasm. 3) Acute RVF: Acute corpulmonale:

Fever.

o

This results from the acute PHTN

Hemolytic jaundice: hemolysis of blood in infarction. o There is manifestations of systemic venous Tachypnea & tachycardia congestion exeept LL edema and ascites because of Lung affection : Acute attacks of dyspnea, chest pain, cough Hemoptysis Lung abscess occur with secondary infection. Pleurisy due to extension of infarction to lung

the acute nature of the condition (unilateral LL edema may be present due to DVT) o Acute RV dilatation occurs (while RV hypertrophy occurs later) 4) Acute circulatory collapse & syncope :

surface:

Sudden onset of stitching chest pain with pleura rub Hemorrhagic pleural effusion

Obstructive shock :

• Acute I COP —+ acute I in cerebral and coronary blood flow

DD from other causes of acute chest pain, acute dyspnea, acute RVF.

o DD from other causes of acute chest pain, acute dyspnea & hemoptysis

DD from other causes of shock: tension

pneumothorax, massive lung collapse, massive myocardial infarction N.B.: > 80 % occlusion of pulmonary vascular bed causing sudden death. 69

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Modified Wells' criteria for pulmonary embolism: ❖ 3 points 1. Symptoms & signs of DVT 2. No alternative diagnosis better explains the illness

❖ 1.5 points 1. Tachycardia with pulse > 100

❖ I point 1. Presence of

2. Immobilization for at least three days 3. Surgery in the previous four weeks 4. Prior history of DVT or pulmonary embolism

hemoptysis 2. Presence of

malignancy

Interpretation : ❖ 6

0 Low probability 0 Moderate probability 0 High probability Investigations:

nbrinogen

FIBRIN

The S1Q3T3 pattern PLASMIN

D-dimer Bad perfusion

Well ventilation

I. II. 1.

Of the cause, e.g. DVT: Duplex US For Pulmonary embolism : Non imaging: B. EGG:

A. Laboratory:

1. Arterial blood gas : low P02,low PC02(due to tachypnea) 2. Increased indirect bilirubin & LDH due to RBCs

hemolysis. 3. Positive D dimer: degradation product of cross linked fibrin by plasmin-mediated protease.

1.

Sinus tachycardia

2.

Right atrial enlargement(P-Pulmonale) j

Right ventricular strain 4. Right bundle branch block 3.

5.

SIQ3T3 pattern:

|

Large S in lead I

I

Pathological Q wave in lead III Flat or inverted T wave in lead III

Imaging : 6. Arrhythmia e.g. AF 1. Chest X-ray: Plump hilar shadow due to distention of main pulmonary artery o Wedge-shaped opacity (Triangular shadow) with the base directed towards the pleura o o Raised copula of diaphragm (due to collapse) ii.

o

Pleural effusion

2.

Echocardiography: chamber enlargement: R.A, RV,Pulmonary A. I.iing scans : ventilation / perfusion scan (V/Q), done by simultaneous : Radioactive gallium 67 gas inhalation & Radioactive 99m Tc- labeled albumin IV . fV/Q ratio: Well ventilation & bad perfusion settle the diagnosis

3. o o

4. Pulmonary Angiography: filling defect in the affected artery. o Recently: Coninutcd Tomography Pulmonary Anglogranhv. gold standard, confirm diagnosis. 5. Spiral (Helical) CT angiography, MRI.

6. Magnetic resonance angiography(MRA): following IV Gadolinium 70

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

■J*

Treatment:

I.

Prophylactic

1. Prevention of the cause e.g. DVT: avoid prolonged bed rest, active leg movement, early ambulation. 2. Prophylactic anticoagulant: • Unfractionated heparin: 5000 SC/ 8h. • LMW heparin e.g. enoxaparin: 40 mg SC/d II. Therapeutic: General : Hospitalization in ICU & resuscitation : Airway & breathing: respiratory support with oxygen up to mechanical ventilation. Circulation : full hemodynamic support with anti-failure e.g. diuretics and anti-shock e.g. dobutamine

Symptomatic : Analgesics : pethidine 50 - 100 mg IV (morphine inhibit RC) Specific treatment according to patient condition : i.

If hemodynamicallv STABLE : further thrombosis

ii.

If hemodynamically UNSTABLE IHF or shockl

& embolisation is prevented by:

A. Thromboiytic therapy :

L Anticoagulants: /clEX^ 1^0 ntv.

to jrtinSRS

Streptokinase t.500.000 I.O-

n

Warfarin 0

•

ia/-: ;V

WC?503r4S?-Ct

satisfe

j

Streptokinase IV: 250,000 IV bolus followed by 100,000 IV infusion /h for 24-72 hours followed by anticoagulants .

B. Trendleberg's operation: Pulmonary embolcctomy: •

Heparin IV initially 80 lU/Kg then infusion of 18 lU/kg/hour for 5 days or LMWH lmg/kg/12hr • Heparin & oral anticoagulants e.g. Warfarin for 5 days • Warfarin alone for 3- 6 months (INR should be 2.5 -3) B. IVC interruption :

Inosxiriin

pulrrwnary art«ry

Pleura!

reflects

Umbrella PJmonary vatve

Embolus

Using cardiopulmonary bypass Performed when thombolytic therapy is contraindicated

Performed when anticoagulants are contraindicated

Done by using IVC ligation, clipping, and insertion of umbrella filter.

71

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Systemic Hypertension(HTN) Definition:

Persistent elevation of systemic arterial BP ; Systolic BP values >140 mmHg and/or Diastolic BP values > OOmmHg At least 2 measures on at least 2 subsequent visits under physical & mental rest.

❖ Grades of HTN by 2018 European Society of Cardiology(ESC)guidelines:

■ o

Category Optimal

o

Normal

o

High normal

130-139

o

Grade 1: mild HTN

140-159

o

Grade 2: moderate HTN

160-179

100-109

o

Grade 3 : severe HTN: hypertensive Crisis

> 180

> 110

"

■

Systolic BP 3 antihypertensive drugs including a diuretic, in optimal doses.

Resistant or Refractory Hypertension

a blocker

o o

Vasodilator

o

o ai and p blocker

CCB

0

Trcatment of complications e.g. acute pulmonary edema

o Enalapril

o 0.5-10 pg/kg/min

Na

Vasodilator

❖ Class o

♦> Dose

❖ IV Drugs:

o

IV drugs :

❖ Definition :

IV.

III.

Anti-hypertensive drugs

1- Angiotensin converting enzyme inhibitor(ACEI)

]

2- Angiotensin II receptor blockers(ARBs). 3- Diuretics.

4- Anti-adrenergic agents : sympathetic blockers 1. Adrenergic receptor blocker:

A. Alpha blocker: prazocin.: best used in hypertensive patient with benign prostatic hyperplasia B. Beta blocker: see before.

C. Combined alpha & beta blocker e.g. carvedilol ❖ Drug 2. Alpha-methyl 3. Clonidine

4. Trimethaphan

5. Guanethidine

6. Reserpine

0 Ganglion

o

0 It deplete

dopa ❖ Mechanism

o

of action

a2 agonist —> Central inhibition of sympathetic system ^ J, BP

blocker

Inhibition of N.A release in

the stores of

postganglionic

NA at the

neurons

o 250-1000 mg t.d.s. orally

❖ Dose ❖ Side effects

nerve

ending 0 10-100 mg/ d orally o Depression, parkinsonis

o 0.1-0.6 mg 0 l-6mg/min. o 10-100 mg/d BID orally. IV orally o Postural hypotension, Bradycardia. o Hemolytic o Rebound 0 Constipation anemia Hypertension o Urinary 0 Chronic Hepatitis o Dry mouth retention

0

0

0 Impotence

CiiThosis

m

Diarrhea

Vasodilator:

1. Alpha Adrenergic receptor blocker: prazocin. 2. CCB: See before

3. Direct vasodilators:

❖ Drug

A. Hydralazine B. Minoxidil C. Diazoxide ♦t* Mechanism of action: Direct smooth muscle VD

❖ Dose

0 10-50 mg

0 25 - 50 mg/d orally

bolus /30 min o

0 50- SOOmg IV

D. Na nitroprusside 0 0.5-10 pg/Kg/min IVl

10-50 t.d.s.

orally ❖ Side effects

o VD: headache, flushing, hypotension with reflex tachycardia which may precipitates angina, syncope. o

Salt and water retention: edema

o

Lupus like syndrome

0

Hirsutism

o Liver disease: Cyanide o o

Hyperglycemia toxicity. Hyperuricemia o Renal disease: Thiocynate toxicity.

Other uses of:

❖ Beta-Blocker

❖ Nitrates

0 0 0 o 0 0

Achalasia & oespageal spasm Biliary colic i Pulmonary venous congestion Hypertensive encephalopathy. Myocardial infarction. Tocolytic in premature labour

o o o 0 0 o

Angina Arrhythmia Hypertension Fallot tetralogy and IHSS Thyrotoxieosis, anxiety, tremors Pheochromocytoma: only after alpha blockers 0 Portal hypertension o Migraine

❖ Calcium channel blockers

o o o 0 o o

Angina and myocardial infarction Arrhythmia Hypertension Fallot tetralogy & IHSS Oesophageal spasm Subarehnoid hemorrhage (Nimodipine)

79

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Valvular heart diseases Anterior snnutus

Anatomy of mitral valve;

Anterior leaflet

AntercKnedial commissure

Posterolateral ^ commissure

Mitral valve is formed of: "Posterior leanet (3 iot)e8}

2 Annulus : anterior & posterior 2 Leaflets (or cusps): anterior & posterior 2 Commissures : anteromedial and posterolateral One central pathway

"■ Chordae tendineae Postsftor-'

2 Papillary muscles with 2 sets of chorda tendinae

annulus

Chorda tendinae are attached to anterior leaflet & posterior leaflet. Mitral valve orifice: measures 4-6 cmL Mitral stenosis

Lateral papillary

Etiology:

muscle

11.

I. Organic 1. Rheumatic heart disease: the commonest , „ 2.

1.

Infective endocarditis

3. Congenital diseases:

//

• Parachute mitral valve

(1 single set of papillary muscle)

\

^—

J

Ik

Medial papillary muede

Functional = relative

Austin - flint murmur : in severe AR

2. t Blood Flow through mitral valve in hyperdynamic circulation 3. Carey Coombs murmur: in acute rheumatic valvulitis.

• Lutemhacher syndrome: ASD + rheumatic MS. 4. Collagen diseases: SLE, RA 5. Cancer: left atrial myxoma.

o 2 years after rheumatic endocarditis stenosis starts

o Significant circulatory disturbance when valve surface area decreased to 1.5 -2 cm^.

Elevated pulm. venous pressure Elevated I. atrlal pressure

> Staging of MS: Hemoptysis

Stage II

Dyspnea

Stage I

Pulmonary

Stage III

conation

Elevated pulm. artery pressure

Edema

^ • L. atrium

enlarged R. ventricle dilated

Stage rv

Hypertrophy Failure Diminished . ventricular

liver

filling ♦

enlar^d tender

Fixed left heart

output

(Ascites)

hypertension)

Porta circulation

Elevated venous ressure

Edema

Systemic circulation

Slight cyanosis

80

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬ Blood vd&sels

Symptoms & signs of pulmonary venous congestion (mention) ± Symptoms & signs of low CO.

cases)

Symptoms & signs

Improvement of pulmonary congestive symptoms Symptoms & signs pulmonary HTN,RVE Symptoms & signs oflow CO. Mitral fades:Malar flush + bluish lips

o LCOP —> low perfusion ^ vascular stasis —> CO2 retention

A. Malar flush due to:

1. 2. 3. 4.

81

In isolated MS: NO LVE,NO cutaneous vasodilation. Pulsus altemans , NO ventricular B. Bluish lips due to LCOP -+ peripheral cyanosis. gallop, NO functional murmur.

LA failure:

Asymptomatic (early

B. I blood flow though mitral valve leading to decrease COP C. t pressure in main pulmonary artery (P. hypertension) with RV hypertrophy

congestive symptoms

A. i in pulmonary venous pressure with improvement of pulmonary

pulmonary arterioles. • Later on permanent sclerotic changes occur. • This pulmonary hypertension leads to:

narrowing, increase in LA pressure is reflected on the valveless pulmonary veins, ending in venous congestion.

Hemodynamics:

Hypertensive MS : t Pulmonary arteriai pressure

Stage III

• Prolonged pulmonary venous congestion leads to hypoxia and VC of

venous pressure

III.

With further mitral valve

Congestive MS: t Pulmonary

Stage 11

MS with LA

II.

compensation

Stage I

• LA enlarges due to stagnation of blood in its cavity • No symptoms occurs

•

I.

❖ Staging of MS:

MS with RV faiiure

stage IV

DVT ->

low CO.

Symptoms & signs of RVF: systemic venous congestion Symptoms & signs of

pulmonary embolism.

RVF

hypertension end in

• Pulmonary

•

IV.

❖ Local Signs: cardiac examination

Silent MS tST

RRRRRRUB

Mid diastolic with

presystolic accentuation

I. • • II. A.

Combined inspection and palpation: over mitral area: Slapping apex = palpable accentuated P' HS

Diastolic thrill ending in slapping apex Auscultation over mitral area: in stage I & II: Heart sounds: t SI

1. Accentuated SI due to:

Closure of rigid fibrosed mitral cusps

Mitral cusps close from the low position to which they are pushed by high LA pressure. Sudden tension of pliable central part of anterior leaflet of mitral valve. Value: the presence of accentuated P' HS exclude: CalciUcation of mitral valve,

b) Presence of significant associated MR in cases with double mitral lesion. B. Additional heart sounds:

2. Mitral opening snap(MOS):

1. Mitral stenosis murmur:

o Site of maximum intensity : best heard at apex o Area of Propagation :locaiized, no propagation o Character: rumbling "R" best heard by the cone of stethoscope. 0 Timing: mid diastolic presystolic o Intensity: presystolic accentuation due to LA contraction, so absent in AF.

o Relation to position of patient: left lateral side & I with exercise. ❖ Silent MS = MS without murmur:

1. J, LA pressure: o Severe pulmonary hypertension, pulmonary embolism, TR, RVF,arrhythmia, tachycardia 2. In association with big ASD : Lutembacher syndrome 3. t LV pressure : LVF,tight AS, HTN. III. IV.

o Caused by sudden downward movement of anterior leaflet.

o Site: midway between left stemal border & cardiac apex o Character: Sharp snapping sound o Diastolic sound following S2 separated from S2 by isometric relaxation phase. • Significance:

• Diagnostic of organic MS • M.O.S. denotes absence of 3 :valve calcification, MR & AF

• Its timing denotes severity: the closer the snap to S2 the severer the stenosis.

• If cusps are calcified & deformed : their movements are

sluggish leading to: a) Disappearance of opening snap b) I SI intensity

In stage III: Signs of pulmonary HTN (mention them) In stage IV: signs of RV dilatation & failure (mention them)

82

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Complications

rr\ LA )LV i

RA ; RV

A. Mitral valve : rheumatic activity, infective endocarditis & calcification of cusps.

R»corrsm

Lsryngoal

B. Left atrium :

1. Outside : huge enlargement causing pressure symptoms on : • Left bronchus : cough - dyspnea

• Esophagus: dysphagia

• Ortner's syndrome: hoarseness of voice due to left recurrent laryngeal nerve compression. 2. Wall:

• Atrial Enlargement with anfiythmias as atrial fibrillation & atrial extrasystoles

o AF : in long standing cases due to atrial stretch, fibrosis & ischemia ^ abnonnal automaticity ^ EFFECT OF AF IN MS :

1. Neck veins : absent a wave with systolic expansion 2. Pulse : marked irregularity 3. Pulsus deficit: more than 10 beats / min 4. Auscultation:

0 Loss of presystolic accentuation

5. o o 6.

o

Variable

0 Disappearance of

intensity of SI

o

opening snap

Complications: Thromboembolisni (percentage of complication increase from 10% Pulmonary oedema ECG: absent P wave, marked irregularity

Loss of S4(no atrial contraction)

40 %)

3. Inside : thromboembolic complications : • Small thrombus in LA : systemic embolization e.g. hemiplegia

• Big thrombus in LA : ball & valve embolus (sudden death) C. Lung:

• Pulmonary venous congestion: cough, recurrent chest infection. Hemoptysis, Dyspnea with its grade (exertional, at rest, orthopnea, paroxysmal nocturnal dyspnea, acute pulmonary edema) • Pulmonary infarction secondary to DVT • Pulmonary Hypertension & RVF ^ MECHANISM OF PULMONARY HTN IN MS:

1. Passive pulmonary hypertension: o Increased pulmonary venous pressure is compensated by increase in pulmonary artery pressure to maintain forward How

2. Vasoconstrictive pulmonary hypertension: o Reflex constriction of pulmonary arterioles may occur to reduce pulmonary blood flow to protect lung from congestion. 3. Obliterative pulmonary hypertension:

o It occurs due to hypertrophic changes in the wall of pulmonary arterioles in response to prolonged pulmonary VC.

4. Obstructive pulmonary hypertension:

o It may occur secondary to pulmonary embolism that results from prolonged bed rest in patients who develop HP

D. Right side of heart: RV enlargement - RVF - functional TR E. Complications of surgery & artificial valves: ❖ Complications of operation: o Arrhythmia especially AF, Systemic embolization o

Traumatic MR, Mitral restenosis

o Post cardiotomy syndrome (Pleuropericarditis)

■

Autoimmune response few weeks after operation due to damaged cardiac tissue, responding to steroids.

❖ Complications of artificial valves: o

Infective endocarditis

o

Thrombo-embolism

o Mechanical dysfunction o Hemolytic anemia o Complications of anticoagulants : bleeding

83

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

PsFTl

^^InvestigatioiM JJ 1. Chest X-ray: L II.

Stage I: no abnormality. Stage II: i.

Posteroanterior view:

«

'is

1. Left atrium enlargement, evidence by: A. Obliterated waist(mitralisation) B. Widening of the carina C. Double contour of right cardiac border

2. Pulmonary venous congestion (Moustache sign) ii. Lateral view with barium : Enlarged LA displaces esophagus posteriorly IIL Stage III & IV: Pulmonary hypertension, RVE,RAE,± calcified mitral valve. 2. ECG:

• Stage I 0

no

• Stage II

• Stage III & IV

•

0

0 RVE: dominant R in VI & deep S in V6. 0 RAE: P pulmonale: tall & peaked p wave

o Absent p wave 0 Irregular QRS

abnormality

LAE:P mitrale : broad &

bifid p wave

Others: AF

Echocardiography : Diagnose lesion ; show stenotic valve

Detect severity: measure valve surface area: In tight MS valve surface area < 1 cm^ Associated lesion : intra atrial thrombi, vegetations of infective endocarditis Detect chamber enlargement(normally < 4cm)& function (motion) Doppler US(CWD)detect transvalvular pressure gradient & valve surface area. Cardiac catheterization and angiocardiography: A. Same value as echocardiography: lesion, severity... B. Right sided catheter is inserted inside pulmonary artery to measure its pressure: o A sharp rise in pressure occur during exercise in cases of MS. o Next, catheter is wedged in a pulmonary arteriole. o This wedge pressure reflects left atrial pressure (Normal= 4-5 mmHg)^ it is t in MS cardiac output

C. Mitral stenosis index

LA pressure

Normally :^x 100= 100%

tu f- ■ ■

Catheter

crude simple measurement of MS severity

it is I in MS

❖ Stage ❖ Degree

0

Minimal

0

Mild

III. Stage III IV. Stage IV 0 Critical tight o Severe

❖ MS Index

o

>50%

0

50 mm Hg.

Valve Surface area < 0.8 cm^(noimal 2.5-3.5 cm^'

II.

treatment.

Indications :

Severe symptoms

N.B.:

mediastinum & calcific wall of aorta

In syphilis huge dilatation with wide

Calcific aortic valve may b4

with LV failure

Pulmonary congestion

LVE.

Wide mediastinum

aortic knuckle

Medical treatment: As MS + {in AR add Antisyphilitic drugs in syphilitic cases : Penicillin.)

Valve replacement in severe symptoms & sings not responding to medical

I.

Boot shaped heart, Aortic configuration: Dilated unfolded

Echocardiography & Cardiac catheterization and angiocardiography:

11.

Pulmonary congestion with LV failure Calcific aortic valve may be seen

Post stenotic dilatation (in valvular cases)

Investigations Chest -X ray

Diagnosis of the lesion, chamber enlargement Detect severity of the lesion by valve surface area and pressure gradient across the valve.

As MS.

o o

LVE & LAE

1.

2.

I.

Peripheral signs of AR: due to big pulse pressure: 1. Head & neck:

1. 2. o 3.

Corrigan's sign : visible vigorous pulsation in carotid arteries in neck. De Musset sign : head nodding due to severe pulsation Other causes of head nodding: cerebellar ataxia, parkinsonism, myoclonic epilepsy & tics. Systolic thrill (carotid shudder): over carotids due to rapid blood flow

; 2. Upper limb: 1. Wide pulse pressure : o Exaggerated difference between systolic & diastolic blood pressure (Normally 30 -60 mmHg) 2. Water Hammer pulse (Collapsing pulse): o Due to rapid upstroke and downstroke of pulse with big pulse pressure.

• N.B.: Mayne's sign: a decrease in diastolic BP of 15 mmHg when the arm is held above the head. 3. Capillary pulsation is demonstrated as follows : o Quincke's sign : in the nail bed : Pressing by finger on patient's nail just to cause blanching , the test is positive when the blanched area becomes alternatively red/blanch with each heart beat o Transillumination by shining a pen torch through lobule of ear o Lighthouse sign: Forehead by scratch, blanching & flushing of forehead, o Becker's sign: Fundus by ophthalmoscope, retinal capillaries are pulsating, o Landolfi's sign: systolic contraction and diastolic dilation of the pupil o Lips by pressure by glass slide to produce blanched area o Muller's sign: pulsating uvula by tongue depressor. 3. Lower limb:

1. Hill's sign :

o Exaggerated difference between SBP in EEs & ULs more than 50 mmHg (nomrally, SBP in LLs is higher than ULs by about 10 - 20 mmHg)

2. Pistol shot(Traub's sign): o Auscultation of a loud booming sound synchronous with each pulse beat over the arteries especially femoral due to sudden distension of collapsed artery 3. Duroziez's sign :

❖ Systolic & diastolic murmurs over the femoral artery if it is slightly compressed with the stethoscope: O Nonnally if we apply pressure to a superficial large artery & auscultate proximally we hear a systolic murmur. O In severe AR we hear both systolic & diastolic mumiur.

O Diastolic M. was thought to be produced by regurgitant stream of blood toward heart in diastole but recently it was proved that it is due to peripheral suction is caused by peripheral VD. Significance of peripheral signs: , ■

.

'

Nonspecific present in hyperdynamic state Determine severity.

❖ Absent peripheral signs in: %

1. Mild aortic regurge 2. J, Systolic BP:

• Associates stenotic valvular lesion e.g. Double aortic lesion with predominant stenosis & MS 3. t Diastolic BP e.g. Systemic hypertension

90

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Mitral valve prolapse(MVP)= systolic click murmur syndrome = Barlow's syndrome = Billowing or floppy valve syndrome

❖ Definition

Prolapse of one or both leaflets of mitral valve (usually posterior one) into left atrium during systole. ❖ Etiology: Idiopathic :

Common in females & may be familial. Due to Myxomatous degeneration of mitral leaflets Represent commonest valve disease among normal (5 % of people) Normal variant 2. 3.

NORMAL

PROLAPSE

REGURGITATION

Valve leaflets close and prevent bacKflow

Valve leaflets balloon upward as tfie ventricle contracts.

blood back into the atnum.

into the atnum.

CT disorders : Marfan's syndrome Cardiomyopathy

Valve leaflets go not

property closo. forcing

Normal closed

Prolapsed

mttral valve

mitrat vaive

Pathophysiology: atrium

1. 2.

Prolapse of mitral leaflets leads to MR,but regurge occur in late systole. Excess stress on papillary muscles with dysfunction & ischemia of muscles

& adjacent ventricular myocardium, with subsequent chest pain & arrhythmia. Click

Clinical picture: A. Symptoms: 1. Asymptomatic in most cases : 2. AiThythmia: Palpitation.

r

JuuL__ SI

Chordaa tendineae

Telesystollc

Papillary ventrida

muscles

82

3. Atypical chest pain: unlike angina is sharp, unrelated to effort, poor response to nitrates. B. Signs: click murmur syndrome:

1. Systolic click or clicks: mid or late systole (sudden tension of prolapsed leaflet)

2. MR murmur follows clicks with whooping character, murmur is usually late systolic (telesystollc) rather than pansystolic.

3. LYE signs in severe prolapse C. Complications: 1. Infective endocarditis

2. Arrhythmias & conduction disturbance.

3. Progressive mitral regurge with LVF

4. Transient ischemic attacks(TIAs)secondary to emholi from roughened surface of valve. InvLstigations: 1. Chest X-Ray: as MR 2. ECG : normal or arrhythmias 3. Echocardiography: diagnostic. Treatment:

2. 3. 4.

5. 6.

Asymptomatic : reassurance may he needed only Antibiotic prophylaxis against infective endocarditis Anticoagulants in AF Aspirin in TIAs

P blocker for arrhythmia & chest pain. Surgical : Vaive replacement: in severe cases (risk of sudden cardiac death) 91

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Tricuspid Regurge

Tricuspid stenosis

AORTA

/

AORTA

9V0

❖ Etiology: I.

Organic:

1. Rheumatic: occasionally with TS

1. Rheumatic: the most common cause, usually

2. Infective endocarditis

associated with MS or with TR

3. 4. 5. o

2. Infective endocarditis.

3. latrogenic: Drugs : Methysergide. 4. Congenital 5. Collagen: SLE,RA. 6. Cancer: Carcinoid syndrome

Ischemic: rupture of RV papillary muscle in MI. latrogenic : Drugs : fenfluramine Congenital: Ebstein's anomaly:

Attachment of septal and posterior leaflets of tricuspid

valve to RV wall rather than to tricuspid ring. 6. Cancer: Carcinoid syndrome. Functional:

II.

o Due to dilatation of RV & tricuspid ring 2nry to P.HTN ❖ Clinical picture Symptoms: I.

0 Due to increased flow across valve e.g. ASD,TR.

1. Of etiology 2. OfLCOP

3. Of systemic venous congestion 4. Palpitation II. Signs: i. I.

General

SVC:

1. Congested neck veins: t JVP with : •

Systolic expansion

Giant a wave

Giant cv wave

• Shallow Y descent

Deep & rapid Y descent

Cyano - icterus or tricuspid facies: Jaundice (hepatic congestion) II.

IVC:

1. Hueelv enlarged tender liver with:

• Presystolic hepatic pulsation (atrial = venous)

• Systolic hepatic pulsation (ventricular = arterial)

•

•

Coincide with S2

• It's due to forcible atrial contraction just

Coincide with SI

• It's due to regurge of blood from RV to IVC during systole

before systole.

2. Hepato-jugular reflux: positive 3. Ascites precox: Ascites precede edema of lower limbs

92

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

ii.

Local

A. Combined inspection and palpation

o Signs of RAE & RVE with hyperdynamic apex. o Systolic thrill over tricuspid area

• Signs of RAE only • Diastolic thrill over tricuspid area

B. Auscultation 1. Heart sounds

o

o

Accentuated SI

Muffled SI

2. Additional heart sounds

o

Tricuspid opening snap.

o

S3.

3. Auscultation of tricuspid area: Murmur of the lesion ❖ As MS murmur but;

As MR murmur but:

o Site of maximum intensity : tricuspid area o Relation to respiration: f with inspiration:+ve

Site of maximum intensity:tricuspid area Area of Propagation :apex & to the right of sternum

Carvallo's sign, being right sided origin.

Relation to respiration: | inspiration :-fve Carvaiio's sign, being right sided origin 4. Others

o Symptoms & signs of pulmonary HTN.

Pulmonary oligemia Pulmonary congestion in MS

o

Murmur of functional TS

Differential diagnosis

❖ Organic TR

❖ Functional TR

0

RA and SVC dilatation

0 Pulmonary oligemia, Calcific valve

o P.hypertension

o

Absent

0

Present

o

o

Present

o

Absent

Thrill

o Digitalis o No effect ❖ Investigations 1. Chest X-ray o RA and RV enlargement o Pulmonary oligemia, Calcific valve

o Improves

2. ECG: o

o RAE with RV enlargement

RAE without RVE

3. Echocardiography & Cardiac catheterization and angiocardiography:: diagnosis of the lesion & its severity ❖ Treatment 1. Medical as MS

2. Surgical

o Valvotomy: commisurotomy o Percutaneous balloon valvuloplasty(PBV): balloon dilatation.

0 Valvuloplasty; valve replacement by a tissue or metal prosthesis

• In patient with mitral valve disease & TR due to pulmonary hypertension, treatment is by mitral replacement • In other cases, tricuspid valve annuloplasty or replacement is done.

93

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Pulinonary stenosis

Pulnionarv Regui ge Etiology: I. Organic:

1. Rheumatic heart disease(very rare)

1. Rheumatic fever (rare)

2. Infective endocarditis. bcaxKh sterxisis

3. Congenital(the most common causeO:

suprsvalviiiar

o Supravalvular stenosis

subvahojlar

valvular

(

o Valvular stenosis(the most common^

2. Infective endocarditis

3. Congenital 4. Carcinoid syndrome 5. Pulmonary commissurotomy

o Infundibular (subvalvular) stenosis

4. Carcinoid syndrome. II.

Functional :

Due to increased blood flow across the valve as in

•

The most common cause that results from dilatation of

pulmonary valve ring as in pulmonary hypertension

PR and ASD.

Dilatation of pulmonary artery above the valve as in

pulmonary hypertension. i* Hemodynamics During systole there decrease in CO along with strain • During diastole there is regurge of blood from in RV leading to RV hypertrophy and finally pulmonary artery to RV leading to RV dilatation and dilatation. finally RV failure During diastole: their resistance to RV filling because

of high end systolic pressure. Clinical picture I. Symptoms:

1. 2. 3. 4.

Asymptomatic in mild cases Symptoms of systemic congestion Symptoms of low CO Symptoms of cause and complication II. i.

5. Palpitation Signs: General

Sign of systemic congestion: • Congested neck veins with giant a wave, Enlarged tender liver •

Edema of LL and ascites

Signs of low CO Arrhythmia

Sign of cause and complication Local

A. Combined inspection and palpation o Signs of RAE & RVE with hyperdynamic apex, Signs of RYE & RVF o Diastolic thrill over pulmonary area Systolic thrill over pulmonary area o Signs of pulmonary hypertension due to pulmonary Percussion: dullness over pulmonary area (post artery dilatation stenotic dilatation of pulmonary artery). B. Auscultation I. Heart sounds Muffled S2

o

Muffled S2

o

Accentuated S2 in pulmonary hypertension

2. Additional heart sounds

S3: over tricuspid area in cases with RV failure S4: over tricuspid area due to powerful RA

S3: over tricuspid area in cases with RV failure S4: over tricuspid area due to P.HTN Ejection click over pulmonary area due to P.HTN

contraction

Ejection click over pulmonary area in valvular stenosis

3. Auscultation of pulmonary area: Murmur of the lesion ❖ As AS munnur but:

As AR murmur but :

o o

Site of maximum intensity : pulmonary area Area of Propagation : tricuspid area Relation to respiration: | inspiration : +ve Carvallo's sign, being right sided origin

Site of maximum intensity : pulmonary area Area of propagation : tricuspid area «&; left infraclavicular area,

o

Relation to respiration: | with inspiration : +ve

Carvallo's sign, being right sided origin. 94

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

4. Others •

Functional TR secondary to RV dilatation ❖ Complications

1. RV failure 2. Infective endocarditis 3. Arrhythmia 4. Pulmonary oligemia may predispose to pulmonary TB.

5. F3 if high RA pressure will force blood into LA —^ opening foramen ovale leading to ASD, here central

cyanosis and clubbing occur later in life. ❖ Investigations 1. Chest X-ray o RA and RV enlargement o RA and RV enlargement o Pulmonary oligemia, Calcific valve o Pulmonary plethora, Calcific valve o Post stenotic pulmonary artery dilatation in o Pulmonary artery dilation in cases with pulmonary

hypertension.

valvular lesions

2. ECG:RAE&RVE

3. Echocardiography & Cardiac catheterization and angiocardiography:: diagnosis of the lesion & its

severity Treatment

1. Medical as MS

2. Surgical

❖ Indications: Done if there is gradient > SOmmHg or

o Valve replacement in severe symptoms & sings not responding to medical treatment.

in cases with infundibular stenosis,

o Valvotomy: commisurotomy o Percutaneous balloon valvuloplasty(PBV): balloon dilatation

o Valvuloplasty: valve replacement by a tissue or

metal prosthesis Congenital heart diseases Etiology :

1. Inherited & chromosomal abnormalities e.g. Marfan syndrome, Kartagner syndrome, Down's syndrome. Turner's syndrome.

2. Infection in the first trimester of pregnancy e.g. rubella(German measles) 3. latrogenic e.g. alcohol & anticonvulsant drugs 4. Irradiation.

5. Immature infants: respiratory distress syndrome of premature infants. ❖ Classifications :

❖ Acyanotic

❖ Chamber affected

o RV hypertrophy

o PS, ASD

0 LV hypertrophy o Biventricular hypertrophy

0 AS,PDA, COA 0

VSD

o 0 0 0

❖ Cyanotic Fallot triology Eisenmenger's syndrome Tricuspid atresia Transposition of great arteries

o

Trancus arteriosus

o Absence of ventricular hypertrophy o Any mild lesion o Fallot tetralogy o

Dextrocardia

How to suspect congenital heart disease? 1) Age < 5 years

2) Associated congenital anomaly 3) Positive family history. 4) Positive consanguinity 5) Cyanosis since or shortly after birth 95

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Atnai septa] defect(ASD).

vena cava

Left atnum

Right

Pulmonary Plethora

atrium Atnai

septal defects: Sinus venosus

iCOP Ostium secundum

Ostium

primum

RBBB

Ventncular septum

Inferior vena cava

RVE -> RVF

Ventricular septal defect(VSD)

Muscular VSD

Perimembranous VSD

Pulmonary Plethora

SVC

Single ventricle

LVE -> LVF

Inspiration

Expiration

Wide physiologic splitting

RVE-^ RVF

Patent ductus arteriosus(PDA) ] L. common carotid

AOB.

Innominate

.rtery

ICOP in LL

artery

— L. subclavian

Pulmonary Plethora

artery

Pulmonary iriery

AORTA

DUCTUS

ARTERIOSUS

, L. Pulmonary V artery LVE ^ LVF

PULMONARY TRUNK

Expiration

Inspiration

Reversed splitting

96

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Potentially cyanotic group with left to right shunt: Ventricular septal defect Atrial septal defect (VSD) (ASD)

Patent ductus arteriosus

(POA)

Description : •

Abnormal communication between 2 atria

Abnormal communication

Ductus arteriosus is a fetal

•

Common associations :

between 2 ventricles.

vessel connecting aorta just distal to origin of left

o Lutembacher syndrome: ASD + rheumatic MS+ Arachnodactyly & high

subclavian artery with bifurcation of main

Arched palate

pulmonary artery or with

❖ Types: 1. Sinus venous defeet(10%):

• Located high in atrial septum near entry of

its left branch.

1. Big VSD: membranous

The normal ductus

defect

arteriosus closes shortly

2. Small VSD: Roger's disease:

SVC.

2. Ostium secondum defect(70%); high

after birth due to sudden

muscular defect

increase in 02 tension

3. Single ventricle : both ASD: membranous & muscular • Involves area of fossa ovalis in midseptal region 3. Ostium primum defect(20%); low ASD: • N.B.: Roger's opening closes spontaneously later on in life • Located in atrial septum near atrio& needs only protection ventricular valves

which may also inhibit prostaglandin E2 synthesis PDA results when the duct fails to close.

against lEC. 1. Blood will flow according to pressure gradient from LA (higher pressure) to RA J,amount of blood passing to LV^ low CO. 2. RA also receives blood from venae cavae —> RA dilatation.

Hemodynamics: 1. Blood flow occurs through the defect from LV to RV

according to pressure gradient during systole. 2. RV is also receiving blood from RA ^ RV enlargement. 3. Increased blood flow through PA(PA dilatation), lung (plethora), and to LA(LA dilatation).

3. Large amount of blood will pass to RV —> RV dilatation, and then to pulmonary artery ^ pulmonary artery dilatation + plethora. 4. Vasoconstrictive and 4. Vasoconstrictive and obliterative changes Obliterative changes in may occur in pulmonary arterioles to pulmonary arterioles to decrease pulmonary plethora —+ pulmonary protect the lung from plethora hypertension. leads to pulmonary 5. Rise of PA pressure increase in RV hypertension. pressure increase in RA pressure with shunt reversal when it exceeds LA pressure 5. Rise of RV pressure occurs & when it exceeds the LV —>• central cyanosis & clubbing pressure ^ reversal of shunt (Eisenmenger's syndrome) with development of central cyanosis & clubbing (Eisenmenger's syndrome)

I. Since aortic pressure is higher than pulmonary pressure both in systole and diastole, there is continuous flow of blood from aorta to

pulmonary artery throughout both phases of the cardiac cycle, this results in:

Dilatation of pulmonary artery.

Lung plethora. LA & LV dilatation. Increased LV stroke volume

—> elevation of systolic pressure. e.

Drop of diastolic blood pressure due to rapid run off of blood from aorta to

the pulmonary artery. 2. Obliteratitive and

Vasoconstrictive changes of pulmonary arterioles to protect lung from plethora —> pulmonary hypertension 3. When pulmonary artery pressure exceeds the systemic pressure —> reversal of the shunt

(Eissinmenger syndrome)

with cyanosis affecting only the lower half of the body (Differential cyanosis). 97

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Clinical picture I.

Symptoms:

1. Asymptomatic in mild cases, symptoms usually delayed until middle age. 2. Pulmonary Plethora: • Exertional dyspnea •

Recurrent chest infections

3. Palpitation 4. Low CO symptoms in big lesion.

5. Shunt reversal (right to left)= Eisenmenger syndrome, effect: a. Blood bypass lung —> central cyanosis & hypoxic clubbing

5.

Shunt reversal: differential

cyanosis & clubbing: in LL

b. iCOP

only {reversed cyanotic

c. J, O2 —»■ 2nry polycythemia—> thrombosis^ pulmonary infarction & hemoptysis

6. Symptoms of systemic congestion in RVF.

6. Symptoms of biventricular

blood enter aorta distal to

Lt subclavian} 6. Symptoms of LVF.

failure

II.

Signs:

i.

General

•

Absent in mild cases

• • •

Bilateral basal crepitation (plethora). Arrhythmia (especially AF). Signs of Low CO in big septal defect.

•

Shunt reversal: Eisenmenger syndrome: central cyanosis & clubbing.

•

•

Signs of RVE& RVF

• •

•

•

Signs of biventricular enlargement with hyperdynamic apex & biventricular failure. ii.

Low CO in LL only (fatigue & claudications). Central cyanosis & clubbing in LL only (differential cyanosis)

Signs ofLVE& LVF. Signs of wide pulse pressure e.g. water hammer pule {]■ systolic BP & J, diastolic BP.}

Local

A. Combined inspection and palpation

❖

Signs of pulmonary hypertension but with wide fixed splitting of S2:

• Signs ofP.HTN but with wide • Signs of P.HTN with splitting of 82. reversed splitting of S2 due to delayed evacuation of • Systolic thrill in left 3"''' and

❖ Wide:

0 t blood flow into RV causing delayed

4"^ intereostal spaces

valve closure

parastemally.

0 RBBB : delayed RV contraction ❖ Fixed (no variation with respiration): 0 S2 does not vary with inspiration because 1 amount of VR during inspiration to Rt side of heart is compensated by an equal diminution of shunt keeping pulmonary

LV. •

Continuous thrill over left

left infraclavicular area.

> N.B.: continuous: systolic & diastolic{blood shunted through duct in both S, D (pressure in A. is 120/80, in P. 25/8)}

flow constant. B. Auscultation

1. Heart sounds: S2 f : P.HTN 2. Additional heart sounds

0 S3 over tricuspid area due to increased

0

blood flow from RA to RV.

S3 over mitral area due to increased blood flow across mitral valve.

0 S4 over tricuspid area & Ejection click over pulmonary area: due to P.HTN 3. Murmur of the lesion

0 No murmur due to passage of blood across ASD (low pressure gradient).

• Site of maximum intensity : left 3'"'* and 4'^ intercostal spaces parastemally • Area of Propagation : all over the precordium.

• Gibson's murmur:

• Site of maximum intensity : 1 and 2"'* intercostal space

• Area of Propagation : left

Character: harsh

infraclavicular area

• Timing: pansystolic

• Charaeter: machinery • Timing: Continuous

•

98

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

4. Others

o o

Functional PR due to pulmonary artery dilatation. Functional PS: due to increased blood flow through pulmonary valve

o

Functional TR: due to RV dilatation.

0

Functional TS: due to increased blood

o Functional MS due to f blood flow through mitral valve.

flow.

o

Functional MR with LV dilatation

o

Aortic regurge: due to prolapse of Rt aortic cusp through high VSD. ❖ Complications

o In ostium primum defect there may be murmurs of associated TR, MR or VSD.

1. Recurrent pulmonary infection 2. Infective endocarditis

3. Arrhythmia especially AF. 4. Paradoxical embolism.

5. Eisenmenger syndrome : reversal of shunt under effect of severe pulmonary hypertension 6. RVF

6. Biventricular failure.

6. LVF

7. Stretch of RV: RBBB.

7. Aortic regurge

7. Aneurysmal dilatation & rupture of the ductus.

❖ Investigations 1. Chest X-ray • Dilated pulmonary artery • Pulmonary plethora • RAE & RVE Fluoroscopy (screen): vigorous pulsation of pulmonary artery at hilum (hilar dance).

•

Evidence of biventricular

•

Evidence of LAE & EVE.

enlargement.

•

Aortic dilatation

•

Evidence of LAE & LVE.

2. ECG:

• RBBB,RAE & RVE

o

Evidence of biventricular

enlargement. 3. Echocardiography & Cardiac catheterization and angiocardiography:: diagnosis of the lesion & its severity ❖ Treatment 1. Medical as MS:

• In PDA add : medical closure of duct by administration of indoemthocin, to prevent PGE2 synthesis. 2. Transcatheter closure of the defect.

3. Surgical closure of the defect.

❖ N.B.: in Eisenmenger syndrome: o Surgical correction of shunt is contraindicated to avoid precipitation of RVF because shunt act as safety valve. o Heart lung transplantation : only radical treatment.

❖ Cyanotic group with right to left $hunt: transposition of great arteries(TGA): Patent Ductus

Description of TGA: 1.

Artertosus

Aorta arises from RV,and pulmonary arteryfrom LV.

2. To maintain life an associated, ASD,PDA VSD should exist. 3.

In addition PS exist to forces oxygenated blood to the left through the shunt. Clinical picture: '

Patent Foramen

1. Central cyanosis since birth ovaie 2. Dyspnea (with difficult feeding), cough, CHF 3. Heart enlarges rapidly in weeks with systolic murmur and gallop Investigations: 1. X-ray: enlarged heart with narrow cardiac base (egg shaped)& pulmonary plethora. 2. ECG: RV hypertrophy 3. Catheter& angiocardiography Treatment:

1. Critical patient without ASD : balloon septotomy 2. Surgical correction for other cases. 99

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Fallot's tetralogy: F4

Pulmonary

O VanMcUar SapUl Daisel 0Puknonaor Stanoala

stenosis

@1HfpartToehy ol Rl.Vamrtole O OvanMnp Aoru

Overnding aorta

interventricuiar

septa! pertropriy

detect

Coarctation of aorta : COA

Patent ductus Left sutx:lavian

arteriosis ^

artery Aortic arch

Narrowed

' (

—aorta

Coarctation

Pulmonary artery Constricted

ductus

Pulmonary artery

Praductal coarctation

Postductal coarctation

Descendlnig aorta Ductus artariosus

Sutrciavlan artery

Sutjscapular artery

Intarcoalal arteries

Inferior

epigastric artery

100

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Cyanotic group with right to left shunt ❖ Failot's tetralogy: F4 ♦♦♦ Coarctatlon of aorta :COA Description:

Congenital cyanotic heart disease with right to left shunt, consisting of: PS usually infundibular = subvalvular: Subvalvular due to abnormal tissue deposition causing right

o Congenital narrowing of a segment of aorta.

1.

ventricular outflow obstruction(RVOO)

The subvalvular tissue is supplied by adrenergic receptors, so the RV outflow obstruction is dynamic and may increase suddenly under adrenergic stimulation. High VSD (v. large): VSD has no murmur; silent: As it is a wide VSD, both ventricles are subjected to the same aortic

2.

Types : Preductal (infantile) type fatal in infancy: subarachnoid hemorrhage Post ductal type : coarctation is below

point of entrance of ductus arteriosus (distal to Lt subclavian)

pressure.

Over-riding aorta: root of aorta is displaced or dextroposed right, arising from both ventricles leading to central cyanosis.

I

RVH; mild because it has 2 pathway: pulmonary artery & aorta. Hemodynamics ❖ Pulmonary stenosis results in:

1. Diminished blood flow to the lower

1. Diminished pulmonary blood flow ^ P. oligemia ^ predispose to

half of the body. 2. Rise of blood pressure in the upper half of the body due to mechanical

IB

2. Increased RV pressure leading to:

RV hypertrophy which is usually of mild degree because of the presence of VSD which presents alternative passage for blood, (ventricle has 2 ways(stenosed P. valve + wide aorta) Rt to Lt shunting of blood across VSD causing central cyanosis.

obstruction of the aorta and due to renal ischemia.

3. LV hypertrophy due to hypertension. 4. Anastomoses develop between the high pressure arteries above the coarctation (internal mammary, periscapular, anterior intercostal arteries) and the low pressure arteries (posterior intercostal arteries) below it.

Clinical picture Symptoms :

I.

Associated congenital anomalies:

1. Central cyanosis & Clubbing:occur since birth or shortly after (late in P' year due to associated PDA) 2. Central dyspnea : hypoxia stimulate RC

Bicuspid aortic valve ^ AS ± AR PDA,VSD

Congenital aneurysms of circle of Willis: Berry's aneurysm rupture: SAH Turner's syndrome (gonadal dysgenesis)

3. Stunted growth

. Squatting position : relive dyspnea & cyanosis: f pulmonary blood flow & so improves hypoxia through kinking of femoral artery leading to f systemic resistance & j, shunt of blood from RV to LV . Cyanotic Spells: Attacks of severe cyanosis & may be syncope Exertion->| sympathetic discharge with adrenergic stimulation of the subvavlular tissue—> spasm of infundibulum of P. artery leading to shunting of unoxygenated blood through the VSD to the aorta-^ severe cyanosis & hypoxia hypoxic syncope (cyanotic spells) It's usually precipitated by exercise, infections or emotional stress If prolonged attack may end with convulsions & death

High BP in upper 1/2 : headache, throbbing (hypertension) 3. Low BP in lower 1/2: weakness, coldness, claudication 2.

Shoulder pain from pulsating intercostal arteries in the back . 5.

Cardiac: dyspnea due to LVF &

angina due to f demands II.

Signs: General

Central cyanosis. Clubbing of fingers. Stunted growth

1. Of association

2. Prominent carotid pulsation & suprasternal notch pulsation 3. t BP in UL, iBP in LL 4. Suzman sign : pulsating intercostal arteries in the back

5. Pulse: radiofemoral delay o Radial pulse : strong o Femoral pulse : weak & delayed

compared to radial pulse. 101

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

ii.

Local

A. Combined inspection and palpation o Signs of LVH & LVF

o Signs of mild RVE

o Pulmonary area is resonant and non-pulsating (mild PS) B. Auscultation 1. Heart sounds

o S2: Accentuated & Single: made of the aortic component only, (t aortic flow & anterior position of ascending aorta)

o o

tS2, S4. Systolic ejection click : dilated aorta & HTN

2. Murmur of the lesioiI

Site of maximum intensity : Best heard in the 2nd, 3rd, 4th, intercostal spaces.

coarctation i.e.

Character: Harsh

a. Lt infraclavicular region (anteriorly) b. Lt interscapular region (posteriorly).

Timing : ejection systolic

• Area of propagation : to A & P area •

Character: Harsh

• Timing : ejection systolic 3. Others

❖ Fallot's Trilogy :F3 o

Valvular PS

Fallot's tetralogy: F4 0 PS usually infundibular

0

ASD

o High VSD (v. large)

o Marked RV hypertrophy • Congested neck veins

o

Over-riding aorta.

o

Mild RVH

Murmur of AS: bicuspid stenotic aortic valve Murmur of AR: dilated aorta

• Neck veins not congested • Cyanosis is delayed • Cyanosis since birth • S2 is weak with wide splitting • S2: single & loud. N.B.: Fallot's Pentalogy: F4 + ASP

❖ Complications

1. Pulmonary oligemia ; leads to pulmonary TB 2. Infective endocarditis. Stunted growth, Cyanotic spells

1. Infective endocarditis 2. LV failure

3. Paradoxical embolism & brain abscess

3. Subarachnoid hemorrhage

4. Polycythemia(from hypoxia): hyperviscocity syndrome (thrombosis)& gout.

4. Dissection and

❖ Investigations 1. Chest X-ray

Wide mediastinum, Dilated aorta Exaggerated waist

2.

Dilated ascending aorta, with double aortic knuckle

Mild increase in Cardiothoracic ratio

Mild RV enlargement; acute cardiophrenic angle with tilted up apex Coeur en Sabot: Radiological appearance of the heart simulates a wooden shoes with elevated tip.

(Pre and post stenotic dilatation called the "3" sign) Rosier signs : Notches in the lower parts of ribs due to pressure of the intercostal collaterals.

2. EGG: Mild RVH

LVH

Echocardiography & Cardiac catheterization and angiocardiography:: diagnosis of the lesion & its severtty Treatment 1. Medical as MS+

Treatment of cvanotic spells :

Squatting position, Oxygen therapy P blockers, morphine to relax the RVOO. 2. Surgical: 1.

Total surgical correction :

Infundibuloseptotomy first: | allow pulmonary flow to dilate hypoplastic pulmonary artery Closure of VSD is done later.

Brock operation:infundibuloseptotomy only Blalock Tussig operation (palliative): creation of a PDA to f

1. Surgical repair of coarctation (caorctectomy with end to end anastomosis) 2. Bypass graft: subclavian flap angioplasty 3. Balloon angioplasty

pulmonary blood flow. 102

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

-■ifc?-

Special murmur According to time:

PDA

MR,TR,VSD

AR,PR

AS,PS MVP,HOCM

SI

MS,TS S2

SI

Systole

A. Systolic :

o Early systolic ■ Very small

o Ejection

B. Diastolic :

o Late systolic

o

(mid)systolic

Pan or

o Early

holosystollc

■

AS

■

■

PS

prolapse (MVP) ■ Hypertrophlc

VSD

Systole

Diastole

Mitral valve

o

Mid & late diastolic =

mid diastolic presystollc

diastolic

■

MR

■

AR

■

MS & TS

■

TR

■

PR:

■

Carey Coomb's

■

VSD

(Graham Steell

murmur:

(functional MS In RF)

murmur)

obstructive

cardlomyopa thy(HOCM) C. Continuous; systolic & diastolic murmur : ■

Patent Ductus Arterlosus

■

Double valve lesion : AS+AR, MS+MR

According to name:

❖ Graham Steell murmur: PR : functional pulmonary regurge in pulmonary hypertension. Roger's murmur:

Gibson's machlnary murmur:

VSD murmur : in Roger's disease

PDA : continues i.e. systolic & diastolic murmur

Carey Coomb's murmur:

Austin flint murmur:

Functional MS : in acute rheumatic valvulitis due to edema of mitral valve

• Functional MS : in severe AR, the regurgitant jet of blood impedes opening of mitral valve in diastole ❖ Cole Cecil murmur:

❖ Sea Gull murmur:

• AR : loud musical diastolic murmur in perforated

AR murmur propagating to axilla

aortic cusps due to infective endocarditis Differential diagnosis between Functional and organic MS: ❖ Functional MS: Austin flint murmur

o No rumbling o

Normal SI

o Peripheral signs of AR .

❖ Organic MS o Rumbling o Accentuated SI, opening snap o Peripheral signs of AR may be absent

Differential diagnosis between AR & PR: ❖ AR

❖ PR: Graham Steell murmur:

o Pulmonary HTN 0 No Pulmonary HTN o t with expiration 0 t with Inspiration o Peripheral signs of AR o Peripheral signs of AR may be absent 103

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Physiology of Conduction System of Heart Atrioventricular

(AV) node Sinoatnal Impulae passes to heart apex: venlrlcuiar excitation begins

SA node gen«rat«a impulM

(SA) node

atrial excllatlon bagins

Venlrlcuiar excitation

complete

Bundle of His

bundle

branch Purklnje

SAnode

Left anterior division

Sinus

Left posterior

Right bundle

A-V node

node \

division

branch

^

A-V bundle Left bundle

Purklnje fibres

Internodal/^-,

1. Sinoatrial(SA) node

/ branch

pathways

i

Right bundle

branch

o Dominant pacemaker of the heart o Located in upper portion of right atrium.

1^' Purklnje fibers

o Supplied by the sinus node artery from right coronary (60 %)or left circumflex (40%). o

The intrinsic heart rate is around 60-100 /min.

o The SAN is activated by sympathetic nervous system and suppressed by the vagus. o Automaticity i.e. Ability to generate impulses. So, nerve supply of the heart aims at regulation of heart rate & not initiation of rhythm. o The atrium is activated from the SAN resulting in a wave in neck veins due to atrial contraction P wave in ECG. 2. Internodal pathways: direct electrical impulses between SA and AV nodes.

I 3. Atrioventricular(AV)node: o Part of AV junctional tissue, only pathway from atrium to ventricle. o Prevent retrograde conduction from ventricle to atrium in case of presence of abnormal ventricular focus —> atrio ventricular dissociation.

o It has a physiological delay, relay station i.e. slows conduction, creating a slight delay before impulses reach ventricles to allow ventricular filling by atrial contraction before ventricular activation occurs. This physiological AV block is useful to protect the ventricle from any tachyarrhythmia arising from the atrium,

o Intrinsic rate 40-60 bpm

"

o Maximum speed of conduction 250 bpm, if more than that, regular block 2:1,3:1,4:1. 4. Bundle of His Q S

o Transmits impulses to bundle branches, o

P Wave

ORS Complex

T Wave

Located below AV node.

5. Left bundle Branch

o The left bundle divides into anterior & posterior hemibundles o Conducts impulses that lead to left ventricle

Aclivation ol(he atria

Activation of the veniiictes

Recovery wave

[6. Right bundle Branch Conducts impulses that lead to right ventricle. 104

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

7. Purkinje system:

o Network of fibers that spreads impulses rapidly throughout ventricular walls, o

Located at terminals of bundle branches,

o Intrinsic rate 20-40 bpm. o

Ventricular activation is presented by:

■ CV wave in neck veins , QRS-T waves in ECG ■ Radial pulse, S1, apex beat and V wave in neck veins o This period of transmission of the impulse from the atrium to the ventricle is presented in ECG by the P-R interval (Normally = 0.12-0.20 seconds). N.B:

1. Vagus supply SAN, atria, AVN but not ventricles: ■ The vagal stimulation decrease the automaticity of the SAN,the excitability of atria and the conductivity in the AVN but does not affect the ventricles.

■

The vagus can be:

o Stimulated by ocular compression, induction of vomiting, cold immersion or carotid sinus massage o Stimulation of vagus nerve causes inhibition of any arrhythmia above AVN o Inhibited by exercise or atropine

2. Respiratory sinus arrhythmia : there is acceleration of HR during inspiration and slowing of HR during expiration: ■ Bainbridge reflex: Inhibition of vagus nerve by inspiration o Inspiration —> increased VR —> atrial stretch —> inhibition of Vagus nerve —> increased HR provided the SAN is the pacemaker of the heart e.g. sinus tachycardia, sinus bradycardia

Phases of the action potential

1 :+ + + + +'

iS i

! 1+ K-

K- ( 1+

i 1+ s.

Fast Na

T_

tl'

'

K*';^±.Na^ i C Na*:ii!:Na^

K* Channel

Channel

EH

1 1+ K* i i+ EO

K* Channels

it

eO

K* Channels

Ca'* K* Channels

0. Phase 0(upstroke): rapid depolarization 1. Phase 1: slow (partial) repolarization

0 This is caused by activation of Na"*^ channels —> rapid Na"^ influx ❖ This is caused bv:

0 Inactivation (closure) of Na"^ channels 0 A starting

efflux

2. Phase 2: plateau or prolonged depolarization

0 Activation of slow Ca^ channels^slow Ca"*^ influx —> Ca"^ influx balances the increasing efflux, so that the membrane

3. Phase 3: rapid repolarization

❖ This is caused bv:

potential is maintained as a plateau for some time.

0 Activation of K"^ channels rapid K"^ efflux 0 Inactivation (closure) of Ca^"^ channels 4. Phase 4: complete repolarization & restoration of the resting membrane potential.

0 This is achieved by increased

efflux

0 The NaVK'^ pump ^ restore the normal ionic distribution around the cell membrane. 105

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Mechanisms of arrhythmia generation I. Disorder of impulse formation: 1. Accelerated automaticity :

Normally there is slow depolarization during diastole.

2.

This mechanism leads to increase the rate of diastolic depolarization or changing the threshold potential. This occurs in sinus tachycardia, escape rhythm (idioventricular rhythm) and accelerated AV nodal rhythm. EAD Triggered activity :

DAD

L Vi .1

o Myocardial damage can result in oscillations of the transmembrane potential at the end of the action potential. o These oscillations, which are called after depolarizations, may reach threshold potential and produce an arrhythmia: A. Early after depolarization(EAD)in phase 3 of action potential B. Delayed after depolarization(DAD)in phase 4 of action potential o This occurs in ventricular arrhythmia or atrial tachycardia induced by digitalis toxicity and exaggerated by catecholamines and electrolytes disturbances . II. Disorder of impulse conduction : re-entry or circus movement: ❖ A prerequisite for re-entry is the presence of two pathways with differing conduction velocities that connect two points: A. The slow pathway: allows slow conduction with a short refractory period i.e. recovers fast B. The fast pathway: allows rapid conduction with a long refractory period i.e. recovers slow ATRIA

ATRIA

Fast pathway

Slow pathway

Fast pathway

l!

Slow pathway

IP 1.

Sequence of events : During sinus rhythm:

Electrical impulses travel down both pathways simultaneously but no arrhythmia is initiated as the slow signal is 2.

terminated when it meets the fast signal. During atrial premature beat:

The impulse is conducted via the slow pathway because the fast pathway is still in refractory period from the previous sinus beat thus producing unidirectional block.

F.fl|»thw.y

O

A,

B. 3.

Shmpa/lhwf Fastpsttmrty

SlowiMllwriiy Fut pathway

SlowpaBiway

This premature impulse will then travel :

Retrograde (re-entry) to the atria via the fast pathway(no longer refractory —>■ recovered) Anterograde to the bundle of His via the slow pathway

Then the cycle is repeated, creating a circus movement whereby the impulse continually cycles around the two pathways, activating the atria retrogradely & bundle of His anterogradely The short cycle length is responsible for the rapid heart rate.

This occurs in cases of paroxysmal atrial tachycardia, atrial flutter and fibrillation & junctional tachycardia 106

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

*1* Clinical ciassification

♦> Tachycardia >100 bpm

❖ Bradycardia

only 2:1, 3:1 or 4:1

It classified into :

30 sec or leads to

hemodynamic collapse

conduction of atrial

impulses to the ventricle, o

regular rate (150-250 / m) Since no retrograde conduction by AVN,

ventricles are controlled by ectopic focus & atria by SAN (complete A-V dissociation)

before terminating into either AF or reverts to

250/min.

Etiology

Ectopic focus in wall of ventricle, discharging at high

b.

Non-sustained: < 30 sec with

o

no hemodynamic collapse Diagnosed if> 3 ventricular premature beats at a rate of

The block may be :

a. Fixed e.g. 2:1

b. Variable e.g. changing

> 120/ min.

from : 2:1 to 3:1 or 4:1

❖ Etiology : Idiopathic (familial), Physiological (very rare), Pathological & Pharmacological causes for all: ❖ Pathological causes: B. Extra-cardiac causes:

A. Cardiac causes:

Hypotension, Hypovolemia Hypo/Hyper-electrolytes Hyperthermia, Hyperthyroidism Hyperdynamic Circulation.

Congenital heart disease Rlreumatic heart disease

Ischemic heart disease

Myocarditis and cardiomyopathy o

1. 2. 3. 4. 5. o

❖ Pharmacological causes: C. Drugs:

Thyroid hormones Digitalis toxicity. Sympathomimetic drugs e.g. Adrenaline. Antiarrhythmic drugs e.g. Class IC

Pulmonary embolism, COPD Wolff-Parkinson-White(WPW)syndrome ❖ Symptoms:

Asymptomatic. Palpitation: see below . Symptoms of Low COP. Precipitation of angina, infarction, HF & syncope. Symptoms of cause e.g. thyrotoxicosis. o Palpitation: rapid, Palpitation: rapid, regular, sudden onset, regular, gradual sudden offset, occurs in onset, gradual offset

o

o Palpitation: rapid, regular or irregular, sudden onset ,sudden offset, occurs in paroxysm,

paroxysm,

o

Polyuria (fAtrial pressure —» ANP).

o

1.

Neck:

1.

o

Systemic embolization .

Palpitation: rapid ,regular , sudden onset, sudden offset, occurs in paroxysm, Sudden death if converts to VF

Signs: 1.

Neck:

A. Neck veins: normal

rapid waves equivalent to radial pulse. B. Carotid sinus

Neck:

A. Neck veins:

A. Neck veins:

o

In paroxysmal atrial tachycardia (PAT): Normal rapid waves In paroxysmal nodal tachycardia(PNT): Regular cannon waves

o

Multiple a waves before

o

a waves: normal rate, 60-

each V wave according to AVN conduction (double

o

v waves: 100-250/min with

rate of pulse) B. Carotid sinus message:

B. Carotid sinus message: no

with each cardiac beats

o

ventricles) 2. Radial pulse:

o

Results in gradual slowing of heart rate which returns to

1.

A. Neck veins:

message:

o

Neck:

100/min

or triple or quadriple the

The pulse decreases in a mathematical fashion

B. Carotid sinus message:

previous rate on release of pressure 2. Radial pulse:

rhythm may occur. 2. Radial pulse:

(150 ^ 100 75/min)& on release of pressure the pulse retums by the same

o

Rate: 100-150/min

o

Rate: 150-250/min

fashion

o

Rhythm: Regular

o

Rhythm :Regular

2. Radial pulse:

o

reversion to sinus

occasional cannon waves

effect(no vagal supply to

o

Rate: 150-250/min.

o

Rhythm: regular.

3.

Auscultation: variable SI with occasional cannon sounds

Auscultation:

3.

Auscultation;

Rate: Variable according

4. Signs of the cause.

accentuated S1

o

In PAT: accentuated 81

to AVN conduction 150 or

o

4. Respiratory sinus arrhythmia:

o

In PNT: regular

100 or 75 / min

cannon sound: it is due to

Rhythm : Regular(fixed block) Irregular (variable block) Auscultation: t SI Signs of the cause.

simultaneous atrial contraction

3.

Present

5.

Signs of the cause

cannon sounds

4.

Signs of the cause

o ■ ■ 3. 4.

N.B. cannon a waves &

during ventricular systole, i.e. atrial contraction against closed tricuspid valve —> marked systolic expansion of neck veins & very loud S1.

108

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

'?♦ Investigations: 1.

ECG:

1.

ECG

1.

ECG

1.

A.

P-wave:

A.

P waves

A.

P waves

A. P waves

•

Rate: 100-150/min

■

Rate: 150-250/min

■

Rate; 250-350/min

■

Rate: normal rate 60-100/min

•

Morphology: normal

■

Morphology:

•

Each P wave is

o

In PAT: deformed

■ o

■ ■

Morphology: normal May not appear, comes

o

B.

followed by QRS-T QRS-T:

Morphology: Deformed morphology replaced by multiple

•

Rate 100-150/min

•

Morphology: Normal

2.

Investigations of the cause e.g.

echocardiography, electrophysiological studies & thyroid

In PNT: inverted P

wave that may: Precede QRS (with short PR interval)

small flutter waves

before each QRST (saw tooth appearance) ■ Multiple P waves before each QRS (2:1, 3:1 or 4:1) B. QRS-T: ■ Rate: 75, 100 or 150/min

Buried in QRS Follow QRS B. QRS-T: • •

2.

function test

Rate: 150-250/min

Morphology : normal Investigations of the

before , after or hidden by the QRS (AV dissociation ) B. QRS-T: ■

Rate: 150-250/min

■

Morphology; broad (wide) bizarre

->

No relation between P &

2.

QRST (A-V dissociation) Investigations of the cause e.g. echocardiography,

■ Normal morphology 2. Investigations of the

cause e.g.

electrophysiological studies & thyroid function test.

cause e.g.

echocardiography, electrophysiological studies & thyroid

ECG

echocardiography, electrophysiological studies & thjToid function

function test.

test.

Treatment: 1.

TTT of the cause,

e.g. Antithyroid drugs for hyperthyroidism Sedative : may be needed

B-Blockers e.g. Propranolol, in severe cases

During the attack:

A. If the patient is hemodynamically unstable —> DC shock for cardioversion ± overdrive pacing. o Ventricular overdrive pacing o Atrial overdrive pacing: are paced at a faster rate than if recurrent VT. tachycardia —> sudden cessation of pacing is usually followed by restoration of sinus rhythm. B. If the patient is hemodynamically stable :

1. Vagal stimulation : carotid sinus massage 2. Rate control = drugs inhibiting AVN; Slow the

1. Lidocaine (first drug of choice):

o

Initial bolus of 2 mg / kg FV, o Followed by maintenance A. Adenosine: of choice 6 -12 mg fV infusion of 1 - 4 mg / min. B. P-blocker (propranolol): 5 mg FV 2. IV Amiodarone, C. CCB ( verapamil & diltiazem); 5-20 mg IV D. Digitalis: 1 mg IV Procainamide, Bretylium Rhythm control - Restore sinus rhythm ( cardioversion a. Chemical cardioversion e.g. Class lA, IC, III antiarrhythmic drugs.

ventricular rate:

b.

Electrical cardioroversion (DC ): if chemical cardioversion fails II.

In between the attacks : Prevention of a future attack :

a.

TTT of the cause

b.

Maintenance therapy: oral drugs: rate control drugs & Class lA, IC, 111 antiarrhjdhmic drugs.

c. Intervention : Ablation of focus : catheter (radiofrequency energy) or surgery Implantable Cardivertor Defibrillator (ICD) (anti -

tachycardia pacing)

Special types of Ventricular tachycardia (VT); WlaiSiMiiiMilMiiiMMllMliaiilMte

o

Torsades de pointes : VT in the long QT syndrome

❖ Accelerated idioventricular rhythm

Polymorphic V.T: ventricular tachycardia characterized on ECG by: Rapid irregular sharp complexes that continuously change from an upright to an inverted position around baseline (twisting of points).

1. Transient ectopic ventricular pacemaker resulting in slower rate at 40-100/min, regular deformed widened QRST complexes similar to those of V. tachycardia 2. Causes: AMI and following thrombolytic therapy (reperfusion arrhythmia). 3. Complication: transient with no hemodynamic

o Prolonged QT interval. 2. Causes: AMI,iK,iCa.

3. Complication: syncope and occasionally to VF. 4.

disturbance.

4. Treatment: atropine is given to accelerate sinus rate and overdrive ventricular rhythm.

Treatment:

A. Treatment of the cause.

B. IV magnesium, P-blocker C. Atrial, ventricular overdrive pacing,ICD D. Left cervicothoracic sympathectomy. 109

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Wolff Parkinson White syndrome:

Delta wave

Delta wave

11

Bundfe of Kei Instead oii die

impolse BBveitoQ tliroiiigh die AV node, it travels down an

accessory pathway

Wide QRS

Short P-R

tC'die ventricles

Interval

Definition :

Pre-excitation syndrome due to additional (accessory) congenital connection (bundle of Kent) between atrium & ventricle and can bypass the AVN. In nonnal sinus rhythm conduction takes place partly through AVN and through the additional pathway —> short P-R interval. It's characterized by : 1. 2.

Short P-R interval(abnonnal pathway) 0.12 sec, due to the presence of delta wave (initial slowing of QRS)

3.

Tendency to paroxysmal tachycardia(abnormal bundle may conduct impulses retrograde) Complications ; Arrhythmia(AF & AV reentrant tachycardia)

1.

DC in hemodynamic instability . Drugs which inhibit conduction in bundle of Kent as Class lA (procainamide), IC (Flecainide), 111 (amiodarone) antiarrhythmic drugs. AVnodal blocking drugs : Adenosine, (3 blocker (propranolol), CCB (verapamil & diltiazem) & digitalis are

Treatment: 2.

3.

contraindicated because they may paradoxically f frequency of conduction in bypass tract leading to f ventricular rate.

Ablative therapy : Transvenous catheter radiofrequency ablation: treatment of choice.

Mapping & surgical excision of bundle by cryosurgery.

Heart Block

• Impairment of impulse conduction at any of the following sites : I.

Between SAN & atria:sinoatrial block

• Failure of one or more of SAN impulses to excite the atria e.g. sick sinus syndrome. II.

Between atria «& ventricles:

• Atrio-ventricular block (AVB); failure of impulses to reach the ventricle from the atria.

• Degrees: 1 degree, 2"'' degree (partial or incomplete), 3''' degree (Complete) AVB. 111.

Along bundle branches:

• Bundle Branch Block (BBB): failure of conduction of impulse in the right & or left bundle A. Right BBB

B. Left BBB

0 Failure of conduction of impulse in the right bundle o

leading to: Wide splitting of the second heart sound

0 Failure of conduction of impulse in the left bundle leading to: 0 Reversed splitting ofthe second heart sound

❖ Causes

• Congenital heart disease, myocardial ischemia, myocardial infarction, myocarditis and cardiomyopathy • RV enlargement: PS, P.HTN, pulmonary Embolism • LV enlargement: AS, AR, HTN IV.

Intraventricular block:

• Resistance to conduction of impulses through the ventricular wall. 110

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Sick Sinus Syndrome - Sinoatrial disease: SAN dysfunction

SINUS NOM

SMUS NOOt

m Hom-

m NOB*

SICK smus STNDBOME

HEABT •U>CK

• Etiology: • Degenerative senile changes of SAN in old ages •

Ischemic heart disease

• Postoperative cardiac surgery • Types: 1. Sinus bradycardia

4

2. Sinoatrial block or sinus arrest: TfTTTT

f Sinus arrest

• Sinus omissions which result in prolonged(>3 seconds)atrial asystole.

• This may be associated with impaired AV conduction & failure oflower pacemakers resulting in periods of ventricular asystole. 3. Tachycardia-bradycardia syndrome:

Atria! flutter

I

»

it Sinus pause

Consists of paroxysmal atrial tachyarrhythmias as AF or flutter which tenninate in prolonged sinus pauses due to suppression of sinus node.

These pauses set the stage for further atrial tachyarrhythmias, in this stage no drugs should be given (only pace maker).

Clinical picture: dizziness, confusion, fatigue, syncope & HF Investigations: Ambulatory (Holter) EGG monitoring Treatment:

Permanent pacemaker implantation Associated atrial tachyan-hythmias may need to be controlled by antiarrhythmic drugs

Anticoagulants: these patients are prone to thromboembolism. Tachycardia - bradycardia syndrome: no drugs needed AVN blockers worsens the bradycardia Heart rate stimulants increase the tachycardia

111

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

t* Sinus bradycardia !

■

-/%■

❖ AV (nodal or junctional) rhythm j

♦♦ Atrio-ventricular block(AVB)

First degree AVB: •4,* 2"° degree: Fixed block

PR = 0.34 second

♦♦ 2 degree: Variable block, Mobitz type I block (Wenckebach phenomena) .

\

^Block

i

X

Dr^ipedbeal

PR X 0.24 sec

PR X 0.24 sec, PR *0.Z8 8e«. PRx0.32:8ec

♦J» 2°'' degree: Variable block, Mobitz type II

AV block at level of

Drapped beat

Orapped beat

bundle of His, or at bilateral

l?Ri*a24 ^. TO x054i8e10 beats / min (weak beats open aortic valve without peripheral transmission of pulse) 5. Auscultation: Variable SI intensity . 6^ Signs of the cause

beats Auscultation:

Normal sounds with occasional irregularity Signs of the cause.

115

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Investigation ECG:

ECG:

P-wave:

Irregular rhythm with PMB followed by

Absent & replaced by irregular fibrillatory waves which may be

compensatory pause:

Supraventricular : Atrial PMB: deformed p wave followed by a normal QRS Nodal PMB : absent or inverted p wave and short P-R interval, a normal QRS

coarse, flue or absent. B. QRS-T:

Rate: irregular Normal morphology Investigations of the cause e.g. echocardiography, electrophysiological studies & thyroid function test

2.

B. Ventricular:

Ventricular PMB:absent p wave , with broad (wide), bizarre (deformed) QRS 2.

Investigations of the cause e.g.

echocardiography, electrophysiological studies & TFT.

Differential diagnosis: between AF & PMB by rate. Rhythm ,pulsus deficit, SI, neck veins, exercise, ECG. Treatment I.

a. Asymptomatic: not treatment b. Symptomatic:

During the attack:

If the patient is hemodynamically unstable -

■ DC shock for

cardioversion ± Atrial overdrive pacing.

c.

Treatment of the cause.

If the patient is hemodynamically stable: Rate control = Slow the ventricular rate by AVN hlockers:

1. 2. 3. 4.

p -hlockers e.g. propranolol CCB e.g. verapamil Stop digitalis or decrease the dose. Anti-arrhythmic drugs e.g. Amiodarone or procainamide.

Indication: when cardioversion fails or is contraindicated.

Drugs: p blocker (propranolol), CCB (verapamil & diltiazem) & Digitalis. Rhythm control = Restore sinus rhythm = cardioversion: Indications :

Age < 65 years old. With Angina or HF.

Absence of atrial thrombus or significant LAE (size ofLA by echocardiography is < 4.5 cm) Absence of embolization or history of embolization Recent onset AF < 1 year. Rapid ventricular rate (150 / min.) despite of treatment Methods of cardioversion :

•

If duration of AF < 48 hrs

Direct cardioversion

• If duration of AF > 48 hrs TEE to exclude left atrial appendage thrombous or anticoagulation for 3 week before and 4 weeks after cardioversion. Methods of cardioversion :

A. Electrical cardioversion

B. Medical or chemical cardioversion

0

0

Structure heart disease; Class III:

0

No structure heart disease; Class IC: Propafenone.

DC shock.

Amiodarone.

3. In failure of drugs: • Catheter radiofrequency ablation of AVN with implantation of ventricular paccmakc. • Maze operation: series of incision in atrium, to prevent the reentry of wavelets responsible for AF.

4. Indications of anticoagulation(INR 2-3): a. Cardioversion (before & after) b. Embolization or atrial thrombus.

c. Valvular AF e.g. Moderate or severe MS d. Non valvular AF with CHA2DS2-VaSc > 2: Condition

Points

C

Congestive HF

1

D

DM

1

H

HTN

1

S2

Prior stroke or TLA

2

Condition

Points

Age 65-74 1 Va Vascular disease e.g. MI 1 Age > 75 2 Sc Sex category: female 1 Interpretation of score: 0: Aspirin 1: Aspirin or oral anticoagulant > 2 oral anticoagulant A2

II.

In between the attacks: prevention of a future attack: as atrial flutter.

116

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

B. Class IB

Phenytoin (epanutin)

o

SVT

o

tachyarrhythmia. WPW syndrome o

WPW syndrome

o

117

PMB

AF

Atrial flutter

Sinus tachycardia

o

o

ventricular

See HE

❖ Used in

VT, VF, V.PMB

Broad spectrum antiarrhythmic.

ventricular

o

o

Used in

arrhythmia e.g.

o

2. Nausea, Hypotension

•

Side effects:

Propranolol Metoprolol.

o o

❖

Atenolol

o

Broad spectrum antiarrhythmic i.e. in supraventricular and

❖ KB.:1, 2, 3: have common side effects as Nausea, Hypotension, Prolong QT interval

mouth

B. Anticholinergic symptoms e.g. dry

A. AV Block

A. SEE like syndrome B. Psychosis 3. Disopyramidc

Convulsion

visual disturbances

Visual disturbance

1. Confusion,

Tinnitus, deafness , 2.

SAN & AVN.

❖ Examples & Side effects:

1. Arrhythmogenesis

Side effects:

Elecainide Encainide

o

Propafenone

o

o

lidocaine :

2. Procainanilde:

o

Minimal effect on AP

(Minimal effect on repolarization)

❖

B. Cinchonism :

•

C. Classic

Marked phase zero depression

Block beta

❖

o

o o

o

o

o

WPW syndrome

Broad spectrum antiarrhythmic

4. Hepatotoxicity

fibrosis

1. Corneal deposits. photosensitivity 2. Thyroid dysfunction (lor i) 3. Pulmonary

9^

.. .^1

l-Ti

amiodarone :

Side effects of

Ibutilide

Sotalol(also pB) Bretylium

Amiodarone

Prolong phase 3 repolarization.

H

adrenergic receptors | ^ cardiac properties (SAN & AVN) o Inhibit phase 4 depolarization in

o

o

K channel blockers

Beta blocker

Class III

111.

Class II:

❖ Mechanism of action :

Act on atrium & ventricle

o

o

Side effects of

treatment.

❖

Tocainide

o

first —> if no

hypersensitivity start

Mexiletene

o

give test dose 1 tablet at

Urticaria & asthma, so

o

•

Lidocaine

o

Site of action:

1. Quinidine: A. Idiosyncrasy :

♦}>

Minimal phase zero depression Shorten AP (J, repolarization)

Act on ventricle only

o

o

Act on atrium & ventricle

o

Moderated phase zero depression Prolong AP (f repolarization)

A. Class lA

According to duration of action potential, they are subclassified into

o

o

Slowing depolarization —> phase zero depression —> i amplitude of action potential

H.

Diltiazem

Verapamil

Prolong phase 2 depolarization.

o

o

o

o

PMB

AF

Atrial flutter

SVT

❖ Side effects: See angina

o

o

o

IV. Class IV Calcium channels blockers

❖ Pharmacological Classification of Antiarrhythnnic drugs(Vaughan - Williams Classification):

Class 1: sodium cluiiintls 11 clscrs: Membrane stabili/ing drugs

o

1.

o

o

AF.

adenosine +

Digitalis: as

flutter

SVT & Atrial

Adenosine:

HF.

effects : see

2. Digitalis : side

Side effects :

Flushing Dyspnea.

❖

Adensoine

conduction

J, AVN

o o

1.

o

❖ Others

1

1

1

;

„„

i Pacemaker !

Definition :

^

.

External energy sources can be used to stimulate the heart, when disorders in

impulse formation and or transmission causing symptomatic bradyarrhythmia. Technique :

Endocardia! wire inserted percutaneous below the clavicle into the subclavian vein or vial femoral vein, inferior vena cava and impacted in the right ventricle under X-ray control and connected to external pacemaker. Types & indications: A. Temporary pacing

B. Permanent pacing

Myocardial infarction associated with:

1. SA disease (sick sinus syndrome) with severe symptoms

Bifasicular block = RBBB + hemiblock

2. Symptomatic l""* degree AVB e.g. syncope 3. 3'"'' degree AVB except if:

Trifasicular block = bifasicular block+ E' degree AVB LBBB

• Asymptomatic

2"'* and 3'''' degree AVB

• Age: Elderly and stable

Only if Symptomatic : Symptomatic Bradycardia despite of atropine.

• Congenital and unchanged 4. Symptomatic bilateral BBB e.g. syncope

Symptomatic AVB Symptomatic BBB

Sudden cardiac death

Etiology : 1.

AVB: Mobitz type 2"'^ & 3'''' grade.

6. Mechanical:

2. AS & IHSS 3. 4. 5.

b.

Rupture heart or aorta Compression : cardiac tamponade

c.

Obstruction :

0

Massive pulmonary embolism

0

Ball & valve embolus in MS

a.

Ventricular tachyarrhythmias Coronary heart disease: myocardial ischemia & infarction Congenital heart disease: Fallot's tetralogy

Pathophysiology: cardiac arrest occur either due to VF or cardiac standstill Clinical manifestations:

J

1. CNS: loss of consciousness, convulsions, brain damage & death 2. Cardiac: absent pulse & heart sounds 3. Chest: absent respiration & cyanosis Management:= Cardiopulmonary resuscitation = CPR Adult basic life support: it is life support without the use of special equipment

Advanced life support: it is life support with the use of special equipment A.

Call for help. Airway: Removal of foreign materials & suction of secretions Head tilt - chin lift,jaw thrust.

B.

Breathing: if apnea confirmed by looking, listening, and feeling

initially 2 breaths are slowly administrated

Mouth to mouth breathing Mask ventilation or intubation and mechanical ventilation

Circulation: IV line & chest compression (100/min, 5 cm depth)- 30:2(every 30 compression give 2 breath). 118

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

D. Drugs used during CPR: • Adrenaline, Atropine, Dopamine, antiarrhythmic drugs (e.g. lidocaine, amidarone), sodium bicarbonate, calcium chloride

E. ECG Rhythm :

1. Shockable rhythm: VF, pulseless VT: DC Defibrillation 2. Non Shock rhythm: continue CPR cycle then reassessment. i. ii.

Asystole. Pulseless electrical activity (electromechanical dissociation):

o No effective COP despite the presence of nonnal electrical activity i.e. QRS without palpable pulse F. Five H & Five T should be excluded :

• • • • • •

Hypoxia Hypothermia Hypovolemia Hypo/hyper electrolytes Hydrogen ion (acidosis) Hypoglycemia

•

Toxins

• Tamponade, cardiac • Tension pneumothorax • Thrombosis,pulmonary embolism • Thrombosis ,coronary artery disease

119

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Vascular diseases

I.

II.

Aq^^eg ments

Diseases of aorta.

Peripheral arterial disease.

rAscending aorta

Diseases of aorta

*t* Anatomical consideration:

Aortic Brcii

❖ Aorta is composed of three layers :

thoracic sertfl Th

!^J

1. Intima

2. Media

AlHtominjii

3. Adventitia

1

tWrareoiil mria

❖ Measurements;

A. 1. 2. 3. B.

{(bdeminBl Ȥrti

Thoracic aorta divides into 3 parts, according to its position in the mediastinum: Ascending thoracic aorta: located in anterior mediastinum, measures 3 cm in width. Aortic arch: in superior mediastinum and gives rise to brachiocephalic arteries. Descending thoracic aorta:lies in posterior mediastinum, measures 2.5 cm in width Abdominal aorta measures 2 cm in width and 15 cm in length, ends by dividing into 2 iliac arteries

L

Aortic Aneurysm

*** Definition: pathological dilatation of aorta. ❖ Description:

Adventitia

Media

Adventitia -

Intima

Media — Intima

Saccular

'

Fusiform

1

False

' aneurysm

True aneurysms

Normal

True aneurysm

Fa lse aneurysm

o According to location: thoracic or abdominal. o According to shape: fusiform (symmetrical dilatation) or saccular (dilatation affecting one wall). ►> Types:

o o

True aneurysm if it involves the three layers of the vessel. False or Pseudoaneurysm: there is disruption of the intima! and medial layers and the dilated segment of the aorta is lined by the adventitia only. ❖ Etiology:

Mycotic ; aneurysm;;

1. 2. 3. 4. 5.

Congenital: genetic predisposition Atherosclerosis (the commonest). Arteritis especially Syphilis Mycotic Aneurysm Medial necrosis e.g. Marfan syndrome

120

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

*** Clinical picture: ❖ Aneurysm of the ascending

❖ Aneurysm of the aortic arch

Aneurysm of the abdominal aorta

aorta Trachea

Heart in

pericardium

Diaphragm

Symptoms

1. Asymptomatic in small cases 2. Visible pulsations in the neck 3. Manifestations of mediastinal

syndrome

1) Asymptomatic in small cases 2) Pressure on the surrounding structures .

3) Rupture of the aneurysm (Usually fatal): o Through the trachea: Fatal hemoptysis

impending rupture) Embolic manifestations e.g. LL

o Through the esophagus: fatal

to LL edema

1. Systolic pulsation and systolic thrill over suprastemal notch, first and 2nd right intercostal space. 2. Diastolic shock may be felt on 2nd right space 3. Apex beat is usually in place( except if associated A.R. causes LV hypertrophy)

ischemia

Compression of the IVC my lead Retroperitoneal hemorrhage or

hematemesis

o Through the pericardium: fatal hemopericardium o External rupture, through the pleura, through the mediastinum A. Inspection & palpation of precordium :

Asymptomatic in small cases Epigastric pain radiating to the back (due to expansion or

Signs Oliver's sign or vascular (true) tracheal tug: Thvfoid i/agusn cncomyroid

hematemesis due to rupture in the duodenum

1. It may arise as a tender expansile mass in the epigastric area. 2. Absent femoral pulsations if there is embolization 3. Dilated veins over the lower abdomen if there is IVC

Cncothyroid

laryngeal n. Thyroid gland

compression

I iusoria I sutKlavian:

B. Percussion :

• Dull 2nd Rt. space & manubrium. C. Auscultation (over Aortic area):

1. Sounds: normal or I 82 + ringing in syphilis 2. Additional: systolic ejection click 3. Murmur: systolic ejection murmur (relative AS)+ early diastolic murmur (relative AR)

Due to adhesions between the

aneurysm and the pretracheal fascia (which is attached to the larynx), there will be conducted pulsations when the larynx is

suspended.

121

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ N.B. CVS of syphilis: ^ 1. Syphilis of aorta :

a. Aortitis which may lead to aortic aneurysm

b. A.R.(valve are healthy due to extension of aortitis to aortic ring causing its scarring, dilatation) c. Coronary osteal stenosis ; with angina pectoris (angina of Lewis) or myocardial infraction All this is due to endarteritis ohliterans of vasa vasovasora

2. Syphilis of pulmonary arteries : pulmonary hypertension (Ayerza's disease) 3. Gumma of interventricular septum ; may produce heart block. r

Investigations: J

1. Chest or abdominal X-ray & screen: •

Dilatation of the aortic outline.

• Calcification of the aneurysm outline may be visible • Marked pulsation of the aneurysm may be seen under screen 2. Trans esophageal echocardiography is the best to demonstrate thoracic aorta while cross sectional ultrasound is the best to visualize and size an abdominal aneurysm 3. 4. 5. 6.

CT, MRI are diagnostic. CT angiography the gold standard Aortic angiography: for severity and extent of the lesion. Investigations for the cause e.g. serology for syphilis Treatment:

1. Medical treatment: to reduce expansion and avoid rupture : • Control of BP and treatment ofthe cause e.g. Syphilis 2. Surgical treatment:

A. Indications:

• • • • •

Rapidly expanding aneurysm (increasing by > 0.5 cm/year) Marfan syndrome > 5 cm Ascending thoracic aneurysm > 5.5 cm Descending thoracie aneurysm > 6 cm Abdominal aneurysm > 6 cm

• Symptomatic aneurysm

• Ascending thoracic aneurysm involving the aortic ring and causing AR B. Methods:

• Surgical excision and grafting

• Endovascular treatment of aortic aneurysms is a minimally invasive alternative to open surgery repair. It involves placement of an endo-vascular stent through small incisions at the top of each leg into the aorta. 122

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Dissecting Aortic Aneurysm

Definition:

❖ Blood enters the media of the aorta and splits (dissects)the aortic wall.

❖ Etiology: ,, 1.

'

.

Dissecting

Dissection

Atherosclerosis.

aneurysm

2. Aorta : coarcitation of aorta, bicuspid aortic valve 3. Blood pressure elevation(HTN)

4. Collagen weakness as in Marfan's syndrome (commonest), Ehler-Danlos syndrome. 5. Trauma.

❖ Pathology: Blood dissects media of aorta (through intimal tear longitudinally) obstructing orifices of branches of aorta. External rupture (fatal) or internal rupture (intraluminal) may occur. Stanford classification : According to location of dissection, divided into: B. Type B or distal type :

A. Type A or proximal type :

o

AI affect ascending, arch, descending o

A2 affect only ascending o Affect only descending

[ ❖ Clinical picture: ❖ Symptoms:

1. Chest pain : Of acute onset Site : retrostemal

Radiation: pain may radiate to the back,jaw or to the abdomen according to the site of dissection. Character: severe sharp stabbing tearing in character and marching in nature. striAe The pain is not relieved by nitrates and usually associated by nausea and vomiting. Acute aortic regurge due to dissection of aortic ring may lead to acute EVP Rupture of the aortic aneurysm may occur either: In the esophagus ^ Hematemesis In the trachea —> Hemoptysis In the pleura —> Hemorrhagic effusion. In the pericardium —> Hemorrhagic pericardial effusion In the abdomen —»■ Hemorrhagic ascites

narrowing

of artery

riBture

Back into the lumen: the condition become latent

The dissection may lead to occlusion of the branches of the aorta leading to: Stroke (carotid artery) Myocardial infarction (coronary artery involvement)

occlusion of

coronary artery myocardial infarction

Unequal pulse and blood pressure either in upper or LL (subclavian or femoral artery involvement) Loin pain and anuria (renal arteries involvement) Paraplegia (spinal artery involvement) Pain in LL and buttocks (iliac arteries involvement) 123

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Signs:

o

Aorta

• Arrhythmia and acute AR

• Pulsating mass in abdomen; expansile pulsation i.e. in all direction i.e. pulsating laterally & antro-posterior . • Discrepancy between carotid arteries pulses, or a difference in BP between two UL or LL. ❖ Investigations: 1. 2.

ECG: done to exclude other diagnoses e.g. pericarditis or myocardial infarction Chest x-ray: may help only in cases of thoracic dissection: Wide superior mediastinum Calcium displacement sign: intimal displacement > 5mm Pericardial or pleural effusion

3. 4.

CT angiography: is the best diagnostic method to demonstrate the aortic flap Echocardiography: It may show: Both the false and true lumen in the aneurysm. The Presence of aortic regurge LV hypertrophy in cases with hypertension Pericardial effnsion

5.

Aortic angiography: for severity and extent of the lesion. Management: i

1. Medical treatment: aim to reduce the shearing forces by:

• I systolic BP to 100- 120 mmHg by Na nirtroprusside or nitrates • I heart rate(50-60 b/min)& J, force of ventricular contraction by p-blockers 2. Surgical treatment: • Replacement of the dissected segment by a synthetic graft •

Correction of AR.

124

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Peripheral arterial diseases 1. Atherosclerotic peripheral arterial disease: Epidemiology

• 6/10000 at the age of40 and increases with age • Sex : in contrast to CHD,women have the same incidence as men

❖ Risk Factors: smoking, DM,hyperlipidemia, homocystinuria. Symptoms: 1. Rest pain 2. Intermittent Claudication

3. Color changes Signs:

1. Absent or weak pulse depending on site of lesion 2. Bruit over stenotic artery

3. Color change of foot and gangrene

4. Dysplastic (Trophic) changes (ulceration, nail changes, loss of hair), and muscle atrophy 5. Diabetic patients differ from non diabetics in the followings: • More aggressive course of the disease • More tendencies for affection of distal tibial arteries

• Associated infection and neuropathy (diabetic foot) Diagnosis:

Evaluation of other atherosclerotic disease: coronary heart disease, systemic hypertension, carotid bruit Differential diagnosis: other causes of pain in the legs: 1. Joint pain & Neuropathic pain 2. Spinal canal stenosis & Venous insufficiency Investigations: 1. Peripheral vascular study :

• Measurement of ankle pressure & ankle/Brachial index : A/B index < 0.9 define PVD • Segmental pressure study to localize vascular disease • Color coded Duplex imaging • Invasive arteriography • Magnetic resonance angiography MRA • Transcutaneous oximetry

2. Laboratory: blood sugar, lipid profile, blood picture. ❖ Treatment:

1. • • • • 2.

Medical therapy : treatment of systemic atherosclerosis: Stop risk factors : smoking, lowering cholesterol, control of blood sugar in DM,control hypertension Antiplatelet: cilostasol (antiplatelet + YD properties), clopidogrel, aspirin Pentoxyphylline : lower blood viscosity and improve microcirculation Exercise therapy: improve claudication distance and delays complications Revascularization by Angioplasty and surgery in case of failure of medical therapy or critical leg ischemia. 125

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

2. Acute arterial ischemia ❖ Embolism

Thrombosis

❖ Etiology : 1. Traumatie following catheterization 2. Hypercoagulable state 3. On top of atherosclerotic plaque

❖ Source of emboli:

1. 2. 3. ❖

latrogenic emboli : following cardiac catheterization Cardiac : valvular heart disease, atrial fibrillation Arterial : aortic aneurysm Site of lower extremity embolization : aortic bifurcation, femoral bifurcation.

• Treatment:

• Treatment:

• Anticoagulation • Catheter directed thrombolysis • Surgical management if anticoagulation fails • Surgery

CiSystemic vasculitides: ❖ Definition: heterogenous group of disorders characterized by destructive inflammatory reaction within the vessel wall.

❖ Classification:Predominantly large, medium & small vessel vasculitides(For more details see rheumatology book)

Vasospastic disease:

❖ A heterogenous group of diseases characterized by intense vasospasm: 1. Raynauds disease 2. Acrocyanosis 3. Frostbite

126

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Hypotension ^0Q

^30

Chronic hypotension BP below normal range;

mmHg.

Types of Hypotension:

1. Chronic asymptomatic hypotension : no signs, no symptoms, need no treatment

1. True chronic hypotension due to LCOP, adrenal insufficiency, chronic bed rest, cachexia & malnutrition . 2. Orthostatic hypotension 3. Neurally mediated hypotension 4. Severe hypotension linked to shock

MyocanJial Anaphyiaxis or valvular disease

kma

Nemogenic impulses

Vaeo^toliiion

1 Hypovdemia -»! SYMI»TOM$ OF SHOCK

Sepsis

mi

Sovera

hypoto^on Hypopeffusion

Cold,etammy skin Edema

ofkesue Thrombosis

I

Cytoidnes

Ceil anoxia

(TNF. IL-1)

Hemorrhage Somnolence,coma

Gastroinleelinai lesions

—[ K/ Oyspntd

ILung feikire(AROS)

Gl bleeding Pwalyticleus Death due to

caidioieqsimiory lalkN>e

127

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Shock = Acute circulatory failure Definition :

o Acute circulatory failure due to hypotension with failure of compensatory mechanisms resulting in hypoperfusion with generalized cellular hypoxia ^ multiple organ dysfunction syndrome(MODs)

]

Etiology

Septic shock :

Infection or any other causes of a systemic inflammatory response that produce widespread endothelial damage with vasodilatation, arteriovenous shunting, microvascular occlusion & tissue edema, resulting in multi-organ failure. N.B.: Systemic inflammatory response syndrome (SIRS)^ sepsis —» severe sepsis —> Septic shock Neurogenic shock:

It is caused by major brain or Spinal injury, producing disruption of brain stem & neurogenic vasomotor control. It may be associated with neurogenic pulmonary edema. 3.

Hypovolemic shock:

A condition causing major reduction of the blood volume e.g. intemal or external hemorrhage, severe bums & dehydration (e.g. diabetic ketoacidosis) Obstructive shock:

Obstruction of the blood outllow e.g. massive pulmonary embolism, cardiac tamponade & tension pneumothorax. 5.

Cardiogenic shock:

6.

Anaphylactic shock:

Any form of severe heart failure e.g. extensive myocardial infarction & acute mitral regurgitation. Due to inappropriate vasodilatation triggered by an allergen e.g.: shellfish, drugs or bee sting.

N.B.: Distributive shock = hyperkinetic shock = hyperdynamic shock = vasoplegic sho^k = low resistance shock include septic, anaphylactic, neurogenic & endocrinal (adrenal insufficiency).

$■

Pathophysiology:

Reduced perfusion to vital organs —> shift from aerobic to anaerobic metabolism | C02 & lactic acid production Areas of hypoperfusion + inllaminatory & clotting cascade. Hypoxic vascular endothelial cells ^ endothelial cell dysfunction —>■ activate WBCs, which bind to the endothelium and release directly : a) Damaging substances e.g. reactive O2 species , proteolytic enzymes b) An inflammatory mediators e.g. cytokines, leukotrienes & tumor necrosis factors. c) Vasodilators : nitric oxide (NO), a potent vasodilator, excess NO is converted to peroxynitrite, a free radical that damage mitochondria and decrease ATP production In septic shock: YD of capacitance vessels leads to pooling of blood and hypotension because of relative hypovolemia. Hemodynamic changes in shock : Type

0

Septic

0

Neurogenic

0

Hypovolemic

0

Obstructive

0

Cardiogenic

0

Anaphylactic

shock CVP COP SYR

Warm

cold

i T i

i i T

i i i

i i t

t i

t

T

t

i t i

128

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Clinical picture:

PaOyPiO^

HYPOTENSION OR VASOPRESSORS

CREATININE,

GiLASaoVJ COMA SCALE I.

PLATELETS

eiLIRUeiN

OLKSiURIA

General features of shock:

A. CNS: drowsiness, confusion, irritability,

B. CVS: hypotension (systolic BP < 100 mmHg), Tachycardia> 100/minute C. Chest: tachypnea >35 D. Oliguria (urine output < 30 ml/hr). E. Multiple ortinii dysfunction syndrome(MODsl = iiuiltlDlc organ failure(MOFi:

CNS: encephalopathy & coma CVS: Myocardial ischemia , depression , arrhythmia Chest: ARE, ARDS

GIT: ileus,, gastritis, pancreatitis, bacterial translocation Liver: ischemic hepatitis & cholestasis Renal: prerenal failure, acute tubular necrosis Blood : DIG, Thrombocytopenia Metabolic: hyper/hypoglycemia, metabolic acidosis Specific features of shock : II. 1. Septic shock :

A. Warm shock: early compensated : fever, warni extremities, rapid capillary refilling. B. Cold shock: late decompeiisated : hypothermia , cold extremities, slow capillary refilling. 2. Neurogenic shock: bradycardia & bypotension. 3. Hypovolemic shock:

o Inadequate tissue perflision: Skin is pale, cold, slow capillary refilling, o

Metabolic acidosis.

4. Obstructive sbock:

• Cardiac tamponade e.g. Pulsus paradoxus 5. Cardiogenic shock:

o Signs of myocardial failure: e.g. congested neck veins, gallop rhythm, basal crepitations, pulmonary edema. 6. Anaphylactic shock:

o o o o o

Signs of profound vasodilatation: warm extremities & low blood pressure, Edema of the face, pharynx and larynx . Erythema, urticaria, angio-edema . Bronchospasm, rhinitis. Nausea, vomiting, abdominal cramp

❖ Monitoring of patients in shock: 1 1. Clinical indices of tissue perfusion:

A. Pale cold skin, delayed capillary refilling & absence of visible veins in the hands & feet indicate poor perfusion. B. Urinary output is a sensitive indicator of renal perfusion & haemodynamic performance. 2. 3. 4. 5.

Blood pressure. Central venous pressure (CVP). Wedged pulmonary artery pressure, by Swan-Ganz catheter. Cardiac output

129

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Investigations: o o o o

Echocardiography, Blood culture, Complete blood picture, Coagulation profile,

Blood gases, acid base state, lactate level, blood glucose, Liver functions , Kidney functions, electrolytes. Management of shock:

• Delay in making the diagnosis and in initiation oftreatment and inadequate resuscitation leads to the development of multiple organ failure(MOF). I.

General measures:

o Patent airway, o Oxygen therapy. o The fluid therapy is given according to the need and according to CV? & type of shock. II. Specific measures: 1. Septic shock: o Treatment of infection by antibiotic(FV) o Surgical drainage for any collections elsewhere. 2. Neurogenic shock: Surgical fixation of cervical fracture 3. Hypovolemic shock: Control haemorrhage & blood transfusion. 4. Obstructive shock: Pericardiocentesis

5. Cardiogenic shock: Dopamine, Dobutamine. 6. Anaphylactic shock: Antihistaminics, Hydrocortisone IV, Adrenaline EM.

130

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Heart Transplantation

❖ Inclusion criteria :

o Refractory end stage heart failure, o

Heart disease class III, IV.

Diseased heart

Donated heart

removed

transplanted in recipient

❖ Exclusion criteria o

Active infection

o Advanced liver or kidney disease, o Irreversible Pulmonary hypertension o

Cancer

o DM with end organ damage. ❖ Complications of cardiac transplantation: o Rejection o Infections e.g. CMV & pneumocystis.

Cardiac involvement in systemic diseases 1. Neurology:

o o 2. 3. 4.

Arrhythmia with autonomic neuropathy Cardiomyopathy with Duchenne muscular dystrophy Respiratory: cor-pulmonale in COPD GiT: liver cell failure hyperdynamic circulation Renal: Chronic renal failure ^ pericarditis, hypertension and arrhythmia.

5. Endocrine;

o o o o o

Acromegaly—> hypertrophic cardiomyopathy & hypertension Pheochromocytoma hypertension and heart failure Thyrotoxicosis ^ arrhythmia, HF. Hypothyroidism—> bradycardia, heart block and cardiomyopath DM —» coronary heart disease

6. Hematology: o

Anemia: heart failure and functional murmurs

o Polycythemia: ischemic heart disease, o Leukemia; pericardial effusion 7. infections: HIV, CMV,IMN and disseminated TB can affect the heart. 8. Rheumatology:

o o o o o

SLE^ pancarditis, Seronegative arthropathies AI. Scleroderma ^ cardiomyopathy, polymyositis. Vasculitis —> coronary heart disease, hypertension . Rheumatoid —> myocaritis ,pericarditis and Al.

Organ involvement in cardiovascular diseases

1. Heart failure: see its complications 2. Systemic hypertension: see its complications 3. Rheumatic and congenital heart disease ^ pulmonary embolism or systemic embolization 4. AF^ embolization.

5. 6. 7. 8.

Atherosclerosis —>■ cerebral, renal, peripheral ischemia . Shock —> multiorgan failure. Dissecting aortic aneurysm renal, peripheral ischemia Infective endocarditis renal, cerebral, mesenterie occlusion.

131

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Nephrology

Topic

Page

oIntroduction

1

oSymptomatology

3

oGlomeruIonephritis

9

oNephrotic syndrome & Nephritic

14

syndrome

o Tubulointerstitial nephropathy

19

oPyelonephritis

20

oRenal vascular diseases

22

oRenal failure

24

o Renal replacement therapy

31

oRenal transplantation

32

o Hereditary renal diseases

33

oElectrolyte & acid base imbalance

37

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Nephrology

Renal arteryBlood with waste

products

RENAL CORPUSCLE

j1

^

i

COLLEaiNG DUCT

DISTAL

NEPHRON LOOP

PROXIMAL

TUBULE

rUSULE

bk>od

Renal vein

Tubular

fluid.

1

Ureter

Arteiioles

'

vteste products

(urine) to the

DESCENDING ^ LIMD

bladder

Mephron

ASCENDING LIMD

Tubule

Detailed

Physiology

Na'K' H.O :

To rena veins

Nutrients

Afferent arteriole Glomerulus

!

Efferent arteriole

Ions HjO : Nutrients

=:>! Na'.Cl- 1

From renal arteryj

Afferent arteriole

Efferent arteriole

Qiomerular capsule convoluted tubule

1. Filtration

Proximat convoluted tubule

2. Reabsorption

Glomeruiar

Collecting tubule

3. Secretion

4. Excretion

Bowman s

capsule

A) Filtration B)Reabsorption C)Secretion

Peritubuiar

Loop of Henle

capillanes

Peritubuiar

Renal corpuscle

AfPtrent-

Renal

Glomeruiar oxpsule

Giemcruloir Space

Unnary excretion

orkHoie

Foot processes

Epithelial cell

Jincto^lonaerutar ceils Basement membrsne

CapiHary iuman

DisWj Convoluted

ProKimoJ fubule

Mesangial call Uesangl^ matrix

GloTMcrulus Macula

Endothelial cell

CafXHones

Dcnsa

PodocyLe

Efferent

orlertole

Bowman s capsule

Parieta!q)ithctial cell Primary Bowman's space

ultrafiltrate Endothelial cell

Podocyte

Podocyte foot processes

Tubular

^tbelial cells Slit diaphraum Capiliary lumen

Mesangial Glomeruiar basemem

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Introduction

The kidney is made of one million nephrons which represent the functional unit. Each nephron is made of:

1. Rcnai corpuscle: Glomerulus & Bowman's capsule

2. Tubule system: proximal convoluted tubule, loop of Henle, distal convoluted tubule & collecting duct. 3. Interstitial tissue

The blood supply of the kidneys about 1300 ml/min i.e. 25% of COP

Normal Urine

• • • •

> Physical properties Volume /day : 1-1 'A liters Specific gravity : 1015 - 1025 Aspect: clear Color : amber yellow

Glucose : nil

> Microscopic • Epithelial ceils :few

•

Acetone : nil

•

RBCs: 0-5 /HPF

•

Proteins: nil

• • • •

WBCs(pus ceils): 0-5/HPF Ova (parasites): nil Crystals: nil or +

> Chemical constituents •

• Reaction (pH): acidic : 5.5 -6.5

• Bilirubin (bile pigment): nil • Urobilinogen : nil or normal trace

Casts : nil or hyaline casts

Function of the kidney Proximal tubule

- Glomeniar

Myoepitheliok^

capilaries

cells with

(cretoiy granges r

Lacis cells

Macula densa

Afferent

Stenoss

arteriole'

1. 2.

Distal eofwoiuted hjtxile

Efferent arteriote

Excretion of waste products & drugs Regulation of body fluids & composition

3. Endocrinai function of the kidney :

c.

d. e.

f.

Rcnin angiotensin system : secretes renin from the juxta-glomerular apparatus for BP control N.B.: At the junction between afferent arteriole and distal tubule there is the juxtaglomeruiar apparatus which is composed of macula densa and myoepithelial cells in the afferent that secrets renin. Secretion of Prostuglandin E2: vasodilator Kinin-kallikrein system: renal blood flow control Activation off Vitamin D3 by one alpha hydroxylase(25 hydroxycholecalciferol 1,25 dihydrocholecalciferol). Secretion of Erythropoeitin hormone . for stimulation of RBCs Degradation of hormone e.g. insulin

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

SYMPTOMATOLOGY Rwui Pain UnKaral Pain-

Pain

? ❖ Causes

1. Renal pain

2. Uretericpain (renal colic)

3. Bladder pain

4. Urethral &

o

o

o Cystitis

0

Inflammation

o Crystals

o

o

Tumors

o

o Suprapubic, with

Stone

o Pyelonephritis o

❖ Character

Bladder distention

Tumor

Acute colicky from loin to groin 0 Nausea, vomiting and sweating

0 Dull aching 0

prostatic pain

Stone

Felt in flanks

o Extend along rib margin to umbilicus

o During micturition

desire to void

o Referred along

o

Referred to

perineum 0 Prostatic pain + urethral discharge 1 by constipation

distal urethra to

vulva, tip of penis

Hematuria

[ ❖ Definition:| o Presence of blood more than 5 RBCs / HPF in urine whether gross or microscopic, o Normally 0-5 RBCs/HPF. ❖ Causes:

1. Prerenai causes:

Coagulopathy: hemophilia, purpura, leukemia, anticoagulants Cyclic hematuria: in women, prominent during & shortly after menses, due to endometriosis of urinary tract.

[

2. Renal causes:

1. Inherited & chromosomal abnormalities e.g. Alport syndrome. 2. inflammation: acute pyelonephritis, SBE, TB 3. latrogenic (drugs): analgesics

Blood dyscroslos

Ronal tumours

Purpuro

Transftionol cell

Sklclo celi trait

carcinoma

Anti-coogulonts

Wilms'tumour

Inrarct

K Tuberculosis

4. Irradiation 5. Immune:

o Acute & chronic glomerulonephritis(GN) o Interstitial disease & Henoch-Shonelein purpura

ureter

H)^>er nephroma Focal ond

6. Renal:

o Renal calculi and crystalluria o Renal masses: hypemephroma, polycystic kidneys o

Stone in

gtomerula neplvitis Neoplasm of ureter

Renal vessels: trauma, infarction, renal vein thrombosis,

Bladder

malignant HTN, vascular malformations. 7. Miscellaneous e.g. strenuous exercise

Tuberculosis

Tumours

Bilhor2ja Prostate

3. Post renal causes:

B^tgn Malignant

Jogger's

1. Ureter: stone, stricture

hoematurio

2. Urinary bladder: cystitis (bilharzial, bacterial), stone, stricture. 3. Prostate : senile enlargement of prostate, tumor

Urethra! neoplasm

4. Urethra: trauma, stone.

'I' Timing :

❖ hiitial hematuria

❖ Total hematuria

❖ Terminal hematuria

o

o Kidney, ureter, urinary bladder

o

Urethra

Bladder neck &

prostate

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Examination for hematuria:

1. General: bleeding tendencies (purpura, ecchymosis) with evidence of cause e.g. tender sternum 2. Abdominal examination:

A. Kidneys:

o Enlarged in Amyloidosis, Polycystic kidneys. Cancer(Hypernephroma), Hydronephrosis, Diabetic nephropathy

o Tender renal angles in pyelonephritis B. Bladder palpation

3. Rectal examination: for prostate e.g. tumor.

i* Differential diagnosis of red urine: 1. Hematuria: prerenal, renal & postrenal causes

2. Hemoglobinuria: all causes of hemolytic crisis e.g. thalassemia & G6PD deficiency. 3. Myoglobinuria: e.g. Malignant hyperthennia, Polymyositis, crush syndrome. 4. Other causes of dark urine:

A. B. C. D.

Diet: coloring matter in sweets, beat roots Dyes: Azobenzene dyes , Phenolphthalein Diseases : obstructive jaundice & viral hepatitis Drugs : Ambilhar, Phenytoin, Rifampici, Niridazol ❖ Investigations i

1. For prerenal: coagulation time, bleeding time, platelet count 2. For renal & postrenal: a) Urine

o Bilharzial ova, pus, casts, sugar, culture o Glomerular bleeding: presence of red cell casts or dysmorphic RBCs b) Sonography & renal arteriography c) Cystoscopy d) Immunological tests: for SLE,PAN,RA.

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Normal

Proteinuria & Albuminuria

Normally: Normal daily urine protein is < 150 mg/day It is a glycoprotein secreted by the cells of the ascending limb of the loop of Henle called Tamm Horsfal! protein with trace albumin.

Range : ❖ 24h urine collection

❖ Proteinuria: protein (mg)/24hr ❖ Albuminuria : albumin(mg)/24hr

o

Normal

0

30-150

o

10-30

0

Micro

o

150-500

0

30-300

o

>500

0

>300

0

>3500

0

No need to check

o Macro (overt) 0 Nephrotic range

❖ Etiology of proteinuria: 1. Over-flow proteinuria:

2. Glomerular proteinuria:

o Multiple Myeloma o Hodgkin Lymphoma

0

Acute and chronic GN

0

0

Acute and chronic PN

0 Fanconi syndrome

3. Tubular proteinuria:

4. Orthostatic proteinuria:

❖ Causes

o

o

Leukemia

Renal tubular acidosis

Cystinosis

o Pregnancy or exaggerated lumbar lordosis

❖ Mechanism o

Increased excretion of

proteins with low molecular weight due to

o Increase glomerular permeability

0

Tubular defect in

protein reabsorption

0 Compression ofIVC by the liver or compression of left renal vein

passing in front of

filtered load >

reabsorptive capacity

vertebral column ❖ Character

o Variable e.g. multiple myeloma: Bence Jones proteins: ■ Precipitate on heating

o It's formed mainly of albumin

o Usual range > 2gm/day

o Is formed mainly of Beta and Delta

globulin.

o Occurs on standing o Normal in supine position

o Usual range < 2g/day

urine to 55°C

• Disappear > 85 °C ■ Reappear on cooling. 5. Renal vein thrombosis & IVC obstruction

6. Sj'stemic causes: Anemia, Burns, Congestive heart failure, Surgery, Exercise, Fever. 7. False proteinuria: pyuria, hemoglobinuria, myoglobinuria, chyluria, excessive mucus especially in females. ❖ 1. 2. 3.

Types of proteinuria: Transient proteinuria : most common, in fever exercise, benign no treatment. Orthostatic proteinuria : benign no treatment Persistent proteinuria: All rest of causes, treatment ofthe cause.

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Clinical picture of proteinuria: o White frothy urine : due to J, surfaee tension of urine(DD yellow frothy urine of obstructive jaundice) o Puffy eye lid & lower limb edema o Clinical picture of cause e.g. nephritic, nephrotic syndrome. ❖ Methods to detect proteinuria :

1. o o 2. 3. 4.

Proteinun

Urinary dipstick test: 2 types Standard urine dipsticks detect macroalbuminuria Albumin-specific urine dipsticks detect microalbuminuria 24 hours protein excretion: normal coagulation of tubular protein

cast formation.

o Normally : 150 mg / day

❖ Types and causes of Casturia:

I

1) Hyaline cast: coagulated proteins in renal tubules o Commonest form and found in nearly all kidney diseases

2) Pigmcnted cast: hyaline cast with hemoglobin or bilirubin . 3) Fatty (lipoid): in nephrotic syndrome

4) Granular casts: in GN,tubulo-Interstitial nephropathy, pyelonephritis, diabetic nephropathy & amyloidosis 5) Broad cast: chronic renal failure 6) Cellular casts:

a) Epithelial (tubular cell) cast: acute tubular necrosis in ARF b) Red cell casts: nephritic syndrome

c) White cell(pus)casts : interstitial nephritis & pyelonephritis

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Urinary Crystals

Q

Ca Oxalate

Uric Add

Triple PhoaplHit

Cystiae

1. Acidic urine:

o Calcium oxalate crystals : envelop shape with hyperoxaluria

o 2, 3. 4,

Uric acid crystals : rhomboid shape with hyperuricosuria and acute urate nephropathy Alkaline urine: triple phosphate crystals (coffin lid shape) Cysteine crystals ; in cystinuria Sulfur crystal# : with sulfadiazine antibiotic White urine

❖ Differential diagnosis of white urine: 1, Urfcosuria

2. Pbosphaturia:clears up on addition of acetic acid 3. Pyuria (Leucocyturia): more than 5 WBCs/HPF,Causes: a) Urinary tract infection b) Acute GN & interstitial nephritis

c) Sterile pyuria (-ve culture): no growth on ordinary culture media despite the presence ofpus ceils in urine. > Causes :

A. B. C. D.

Antibiotic therapy Prostatitis & Polycystic kidney NephroCalcinosis Drug induced interstitial nephritis .

E. Non-infectious conditions; Cancer ofthe renal tract, renal Calculi & Catheterization

F. Infection by unusual organism e.g. tuberculosis, mycoplasma & Chlamydia trachomatis G. Graft rejection

4, Chyluria: presence of fat in urine in filariasis ,fat clears by addition of ether Urine output(UOP)

0

Normal

o

1-2

o Polyuria o > 2-3 L /day

liter/day o Causes: see diabetes insipidus

0 Oiiguria o < 400 mFday

o E.g. ARE, acute GN & terminal cases of

o

Anuria

o Complete absence of UOP > 12-24hrs e.g. Post renal causes ofARF

CRF

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Polyuria: UOP > 2-3 L /day

❖ Etiology:

I.

Psychological: compulsive water intake : Psychogenic polydipsia

o Psychogenic polydipsia should be differentiated from diabetes insipidus: A. Psychogenic polydipsia 1. Plasma osmolarity

B. Diabetes insipidus(DI)

0 J,(over-hydration)

o

0 i

o r o i

t

2. 8 hours water deprivation test: 0

Urine volume

0 Urine osmolality 3. Exogenous ADH

0 t 0 No improvement

o Improvement in cranial DI

o Treatment is by sedative & hypnotic 11. Physiological (functional): high fluid intake : water, tea, beer ater, coffee, cotii III.

Pathological:

A. Renal causes:

1. Stage 1 chronic renal failure 2. Diuretic phase of acute renal failure

I

3. Tubular disorders : chronic interstitial nephritis, renal tubular acidosis ,Fanconi syndrome 4. Others: Hypokalemia, Hypercalcemia B. Endocrinal causes:

1. Diabetes insipidus 2. Diabetes mellitus

3. 4. 5. IV.

Thyroid: thyrotoxicosis Parathyroid : hyperparathyroidism and other eauses of hypercalcemia Adrenal: Addison's disease. Gushing syndrome. Conn's syndrome, Pharmacological:

A. Excess diuretics

B. Drugs causing nephrogenic diabetes insipidus:

o

Lithium, Demeclocycline, Methoxyflurane anesthesia

C. Drugs causing polydypsia(dry mouth): o Atropine, Chlorpromazine.

V.

Miscellaneous: transient polyuria occur after attacks of: migraine. Epilepsy, SVT

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Glomerulonephritis(GN) Deflnition

Immune mediated glomerular injury with symmetrical simultaneous involvement of both kidneys at the same time. Etiology: "t* Causes of glomerulonephritis: (Causes of iieplirilic syndrome Causes of nephrolic syndrome

I.

Primary GN: Idiopathic GN: according to histopathology; see later. II. Secondary GN: 1. Inherited GN (congenital) e.g.

Congenital Nephritic syndrome

o Alport's syndrome: nephrotic sydrome, nerve deafness ± cataract.

o Sickle cell disease , Fabry's disease 2, Infections:

o Subacute bacterial endocarditis(SBE) o Hepatitis B & C, HIV o Malaria, Schistosomiasis & Syphilis

o Post streptococcal GN: ■ The most common cause, it is immune complex

disease due to infection with nephrltogcnic group

A |l-hemolytlc streptococci (type 1,4,12 in acute ■

tonsillitis & type 49 in pyoderma), Ag-Ab complexes deposit in the GBM,activate

complement and initiate inflammation. 3. Irradiation

4. Immunological:

o Cryoglobulinemia & Collagen diseases: RA,PAN,SLE, Scleroderma. o

Serum sickness & Snake venom

o Henoch-Schdnlein purpura & IgA nephropathy (Berger's disease) Hemolytic uremic syndrome(HUS) Thrombotic thrombocytopenic purpura(TTP) Wegener Granulomatosis Good pasture syndrome

Churg-Strauss syndrome S. latrogenic: Nephrotoxic drugs:

o Heavy metals: Lead, Mercury, Heroin o Drugs: NSAIDs, Allopurinol, Penicillin, Penicillamine, Captopril, Sulphonamides, Hydralazine, Rifampicin, Gold. 6, M etabolic : DIABETES MELLITUS & Amyloidosis 7, Mechanical (renal congestion): o

Renal vein thrombosis

o rVC thrombosis, constrictive pericarditis, congestive HP. 8, Malignancy:

o Leukemia, Lymphomas, Multiple Myeloma. 9, Miscellaneous:

o Accelerated HTN,

o Preeclampsia, o Post renal transplantation o Reflux nephropathy (vesicoureteric reflux)

o Unilateral Renal artery stenosis.

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Mechanism & pathogenesis of GN:

t Abnormal activator C3NF

; Irnmuna1

coirpitx

; naulrophil] I Aggrsgatad

I platalata

% Chtmo*

Formation of

Ralaaaa of anzymaa;

J. Normal inhibitors

miaolhrombus

1. Anti-glomerular basement membrane antibody(anti-GBM):

o Formation of antibodies against BM ofthe glomerulus and lung (antigenically identical),

o E.g. Good pasture syndrome: Heinaturia(RPGN)+ Hemoptysis 2. Immune complexes (antigen-antibody) deposition: A. Formation of immune complexes: antigen antibody complexes: ❖ Antibodies

❖ Antigen

0 IgG or IgM

o Exogenous e.g. Streptococcal infection 0 Endogenous e.g. host DNA in SEE B. Circulating immune complexes are trapped by gioiiierular basement membrane(GBM)—> complement

activation —> release of anaphylatoxins C3a and C5a which : o React with receptors on mast cells and basophils causing release of vasoactive amines e.g. histamine which increase vascular permeability. o C5a is chemotactic for neutrophils which engulf the immune complexes, degranulate and release iysosomai enzymes that destroy the basement membrane. C. Piatciets aggregation: they release vasoactive amines and form microthrombi which cause local ischaemia and further tissue damage. o Immune complexes(ICs) appear by electron microscope as lumps in GBM. 3. Abnormal complement activation;

o Absence of normally occurring inhibitors.

o Abnormal activators as C3 NF (nephritic factor) is seen.

TCell

4. T-cell dysfunction: minimal change nephropathy & post renal transplant rejection

5. Intravascular coagulation:

o Henoch-Shonlein purpura o Hemplytic uremic syndrome o Thrombotic thrombocytopenic purpura.

6. Accumulation of abnormal metabolites: DM & amyloidosis 7. Abnormal glomerular structure: abnormal collagen, abnormal foot process or basement membrane.

10

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ N.B.: Sites ofimmune complexes deposition in the giomerulus: ^ ^I Subepitheli^

Intra membranous

4 Subendothelial

1. Sub-epithetical humps 2. Sub-epithetical spikes 3. Sub endothelial

4. Mesengial 5. Basement membrane

❖ N,B.: Focal proliferative GN :IgA nephropathy (Berger's disease)

o Is the most common glomerulopathy worldwide o Due to deposition of IgA + C3 in giomerulus.

o Common in young adults, presented with recurrent hematuria within 2 days of upper respiratory tract infection (synpharyngitic GN) o Primary IgA nephropathy associated with Henoch-Schonlein purpura. o Secondary IgA nephropathy e.g. due to chronic liver disease, celiac disease, ankylosing spondylitis

11

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Histopathology: Minimal change disease

Membranous GN FSGS

Fusion of

foot processes Basement membrane normal

HVMWOSIS

morpholcgicaHy

Focal proliferative GN

Mesangioproliferative GN

Mssangial cell proliteration

Membranoproliferative type I

Membranoproliferative type II

Some fusion of

Some fusion of

foot processes

foot processes

SplK or doutte Dense rH)boni8(e

basemem membrane

deposit in basement memt>rarM

Subendothelial deposits

Light necroscopy «Thickened basement membrane

Ugtit mtcroecopy •Spfit basement membrane •MesangiaJ cefl proliteration

•Mesangiai ceil proliferation

Rapidly progressive glomerulonephritis(RPGN)

B Diffuse proliferative GN

Epftheliel cefl

Glomerular tMsement

membrime

'Hump' Blood space

Endothetial ceil

12

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

I.

Minimal

o

Fusion of foot

o

o

Focal(some glomeruli affected) & segmental (part

o

IgM-C3 deposits

hyaline material

replacement of glomerulus by

is affected)

of each glomemlus

Nonnal

o

2. Focal segmental glomemlosclerosis

o

o

o

3.

IgG-C3 deposits

subepithelial spikes

&

Thick GBM

Thick GMB

GN

o

o

o

o

encroachment

IgA-C3 deposits e.g. IgA nephropathy

o

Wide variety e.g. IgG or IgM - C3 deposits

o

o

o

o

autonephritic factor) Type III: as I or II

intramembranous(low C3 with circulating

Tvpe 11(Dense deposits disease): C3 NeF

and subendothelial.

Tvpe I: IgG + C3 deposites in mesingium

similar to type I & II.

Tvne 111: Morphologic variants with features

deposits,

intra membranous

disease): mesengial &

Tvpe 11 (Dense deoosits

deposits,

o

Tvne I: mesengial &

mesangial matrix with capillary lumen

o

ANCA: +ve ANCA: -ve

■

could be:

no Ig deposits,

Tvoe IIKpauci immune): few or

granular deposits

Complex): Ig

Tvpe II(immune

Ig linear deposits

Tvpe 1 (anti GMB antibodv):

formation

with crescent

Extracapillary proliferation

capillaries

■

o

o

o

o

capsule forming

duplication (Tran-like appearance).

subendothelial

Marked proliferation

Bowman's

GBM splitting or

cresents around

cells of

cell proliferation with

Proliferation of

partial epithelial

o

matrix & endothelial

Mesengial, mesangial

of mesangial cells &

C. Immunofluorescence(IF)

of mesangial

proliferation

Focal

B. Electron microscopy(EM)

glomerulus)

of

❖ Microscopic examination: A. Light microscopy(LM) o o Marked proliferation Focal proliferation of mesangial cells & mesangial matrix with of capillary lumen mesangial encroachment cells(< 50%

o

o

o

IgG-C3 deposits

Subepithelial immune deposits (humps)

mesengial, endothelial,& epithelial cells

Proliferation of

II. Proliferative GN: t number of cells in the glomerulus GN presenting with nephritic syndrome o GN presenting with nephrotic, nephritic, or mixed syndrome 2. Diffuse 2) Mesangioproliferative 3) Membranoproliferative 1. Rapidly 1) Focal proliferative GN progressive GN (Mesangiocapillary) GN proliferative (cresentic GN) GN GN

13

Chronic G.N.: End stage of all GN,CRF develops slowly over years, Microscopic examination: combination of sclerosis, membranous & proliferative. GN may presents as asymptomatic proteinuria or microscopic hematuria, nephrotic syndrome, nephritic syndrome, ARF& CRF.

complexes deposits)

immune

-Ve(no

processes of podocyte

Normal (nil)

(Nil svndrome

change disease

Membranous

Non-proliferative GN

GN presenting with nephrotic svndrome:

o

1.

o

Pathology: Pathology is determined by renal biopsy examined by: Light microscopy(LM),Electron microscopy(EM),Immunofluorcsccncc (IF):

•** Nephrotic syndrome Podocytes

Basement Membrane

D,amaged

, Damaged

Bioed

Atbumfn

Negative tons)

glomendar barrio-and

Proteinuna

Hypoalbuminemia

resistance

Increased open

channel probability of ENaC channels

Insufficient

Increased

osmotic pressure

number and

for fluid resolution

activity of Na/K ATPase

Loss of:

Immunoglobulin, Hyperlipidemia

Antithrombin III & Protein C & S Cholecalciferol Transferrin

Thyroxin binding protein

V Nephritic syndrome

afrti^en^Mtibody

Azotemia & Uremia Hematuria

Proteinuna

Oliguria

Pallor + Oedema

14

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Nephritic syndrome

Nephrotic syndrome ❖ Definition

o Heavy proteinuria > 3.5gm/1.73m^/day.

❖ Nephritic syndrome is a clinical syndrome characterized by an acute onset of: o Hypertension, Pallor, Odema,

o Hypoproteinemia

o

Nephrotic syndrome is a clinical syndrome characterized by gradual onset of:

Hematuria, Proteinuria, Oliguria.

o Hyperlipidemia & lipiduria with lipid cast. ❖ Etiology & pathology I

I.

Primary: Idiopathic:

Minimal change GN (the commonest cause in children) Membranous GN (the commonest cause in adults) o Secondary: see before. II

o

1. Clinical picture of the cause e.g. DM.

Primary :Idiopathic:

o Membranoproliferative GN or diffuse proliferative

II. Secondary: see before Post streptococcal GN: the most common cause Clinical picture ❖ Pathogenesis «& clinical picture: o

2. Proteinuria :

1. Clinical picture of the cause e.g. child with history of

• Pathogenesis: Structural changes in GBM

Tonsillitis

Electrophoresis differentiates between : Selective proteinuria:

II.

Non selective

proteinuria

Only LMW (albumin, ai

o

Both LMW &HMW

as a2, P, Y globulin

globulin & transferrin)

especially IgG In minimal change disease

J, Negative charges in glomerular capillaries which normally repel

o

In membranous

nephropathy

2. Hematuria: gross (tea - colored urine, smoky urine) or microscopic. 3. Proteinuria: Sub-nephrotic range
Clinically:

Frothy urine. Protein energy malnutrition (-ve nitrogen balance) 3. Hypoproteinemia due to :

❖ Pathogenesis: o Anorexia & mal-absorption from GIT edema o

❖ Pathogenesis: o GN occurs after 1-3 weeks, the latent period is needed for formation of immune complexes & their deposition in glomerulus.

❖ Pathogenesis: Glomerular capillary injury

4. Oliguria(UOP < 400 ml/day): ❖ Pathogenesis: ■ I GFR due to obstruction of glomerular capillary lumen by: o Proliferating glomerular cells. o Infiltrating inflammatory cells. o

Proteinuria

substances

o t Catabolism of protein in kidneys ❖ Clinically: A. (.Albumin: o

Intra-renal VC due to imbalance between local VC

(t) e.g. leukotrienes & local VD (J.) e.g. nitric oxide ❖ a) b) c)

Edema

o Wasting of muscles. o White nails (Leukonychia)

o I Total Ca^^ ^ tetany & hypocalcaemia.

Complications : ARE Acute uremia & Azotemia(j urea & creatinine) Hypervolemia Hyperkalemia with metabolic acidosis

5. Hypertension :

B. Loss of:

❖ Pathogenesis: j, GFR t Renin —» j Ang II: o VC —>■ hypertension. o t Aldosterone —> salt & H20 retention —> HTN &

1) Loss of I gamma globulin (Antibodies= Ig): ■ Recurrent infection especially with streptococci, haemophilus & pneumococci causing abdominal crisis due to peritonitis. 2) Loss of Anti-thrombin III & Protein C «& S (Natural anticoagulants)& t I'ver synthesis of clotting factors —> hypercoagulability & thrombosis e.g. renal vein

edema

❖ Clinically: ■ B.P. ishigh(> 160/ 100). ■ Severe hypertension may lead to HF, pulmonary edema, encephalopathy, papilledema & retinal hemorrhage.

thrombosis

3) Loss of Cholecalciferol binding globulin —>(, vitamin D i Ca^"4) Loss of Transferrin Fe ^ microcytic hypochromic Anemia. 5) Loss of Thyroxin binding protein —> J, total T4 & T3 but normal free T4 & T3 (false myxoedema)

6. Pallor : due to anemia and generalized VC (pale HTN)

❖ Pathogenesis: I Erythropoictin —♦ anemia.

15

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

4. Massive edema:

❖ Pathogenesis: 7. Mild Edema: 1. Underfill theory : A. I Albumin —> I osmotic pressure of plasma protein —> ❖ Pathogenesis: fluid shift from intravascular to interstitial compartment " Primary salt retention due to glomerular capillary —+ edema underfill of intravascular volme (J, IVV) injury —> proteinuria & oliguria (J, B. Hypovolemia (J, IVV) —>■ + RAAS —> f aldosterone GFR—>taldosterone) —> salt & water retention —> C. Hypovolemia (J, IW) ^ f ADH & J. atrial natriuretic edema peptide (AN?) ❖ Clinically : B & C ^ secondary salt & water retention —> a. Course: aggravates edema o Early: migratory i.e. edema starts gradually in loose ii. Overfill theory: areolar tissue in the periorbital region (eye puffiness) ■ Frimarv sodium retention due to : in the morning then LL edema at the end of the day. a) 1 activity of epithelial sodium channel in the b. Character: bilateral, symmetrical, soft pitting edema collecting duct b) Increased activity of Na-K ATPase c) Tubular resistance to ANP

Primary sodium retention —»intravascular overfilling (hypervolemia) ^ f hydrostatic pressure —> excess fluid spilling into the interstitial compartment ^ edema Clinically: a.

Course;

o

Early: migratory

b.

Later on generalized anasarca i.e. ascites, pleural effusion, pericardial effusion Character as nephritic syndrome

c.

Complication: j IW ^ ARF & Shock.

o

5.

Hypeiiipidemia: Pathogenesis:

Hypoalbuminemia

stimulate synthesis of cholesterol

& albumin in liver.

Loss of lipase enzyme as a part of proteinuria Clinically : o

Acceleration of atherosclerosis

in urine:lipiduria & lipid east(characteristic) 6. BP normal but may be)if: Original disease predispose to HTN e.g. DM,SLE o Complicated cases with atherosclerosis or renal failure. o '> Differential diagnosis: 1. Of cause & clinical picture e.g. proteinuria 1. Of cause & clinical picture e.g. Hematuria 2. From other causes of generalized edema 2. From other causes of generalized edema 3. From nephritic syndrome & myxedema 3. From nephrotic syndrome o

1. Urine analysis: o

Volume : normal

o Aspect: frothy due to proteinuria o Specific gravity: high

Investigations Urine analysis: Volume : Oliguria: if < 400 ml /d = acute RF Aspect: smoky or dark urine (gross hematuria)

Specific gravity: high

o Proteinuria > 3.5gm/l.73m^/day, selective & non-

Proteniuria :subnephrotic < 3.5 gm/1.73m^/day Microscopic examination : RBCs & RBC casts (characteristic) Blood investigation: normocytic normochromic anemia, potassium may be increased. Kidney funetion tests: i GFR, I creatinine clearance I Creatinine & blood urea

selective

o o 2. o o o o

Lipiduria Microscopic examination: lipid casts (characteristic) Blood investigations: Hypoproteinemia : J, total protein, J, albumin i Fe, Ca, K, totalT4 &T3 Hyperlipidemia: t cholesterol & triglycerides t Clotting factor level.

Renal imaging :PUT, US, CT: mild swelling of the kidney Renal biopsy for pathological type. Investigations for cause in secondary cases: 1 ASOT & -i-ve ASZ test: streptococcal infection

3. Kidney function test: normal except if RF occurs. 4. Renal imaging: PUT, US,CT: mild swelling ofthe kidney.

5. Renal biopsy for pathological type. 6. Investigations for cause in secondary cases: e.g. DM. 16

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Treatment

I. II

i.

Complete bed rest.

ii.

Dietary management & symptomatic treatment:

o

1)

Salt & fluid restriction in severe edema & hypertension Fluid restriction: volume of urine/day + 500 ml

0

insensible loss + 500 ml/l° C fever. o ■

Drugs for edema : diuretics

o

o

o 0

3.

4.

Aldosterone antagonist(spironolactone) in resistant

urea Protein: normal protein intake is advisable 3) CHO: excess carbohydrate in diet to avoid N.B.: high protein diet may increase the proteinuria endogenous protein catabolism Drugs: IV Albumin to raise plasma osmotic pressure CHO: excess carbohydrate in diet to avoid endogenous 4) Fats: avoid excess fat in diet because they are nauseating. protein catabolism

5) Vitamins & Minerals: K restriction in ease of hyperkalemia o

Fats: avoid excess fat in diet to correct the

hyperlipidemia & they are nauseating 0

5. 0 a.

b. c.

Drugs : slmvastutin

o

iii.

Vitamins & Minerals:

K supplements for hypokalemia which result from: 1 intake from anorexia, nausea & vomiting J, absorption from GIT edema Secondary hyperaldosteronism

o

Iron supplements in case of anemia. Treatment of cause: e.g.

In post streptococcal GN: Benzyl Penicillin: I million

U/6 hrs for 10 days. Treatment of complications e.g. 1) Hypertension: antihypertensive drugs e.g. Hydralazine or Alpha-methyl dopa iv.

d. Diuretics & steroids. o

Drugs for edema: diuretics:

Loop (furosemide) or thiazide diuretics. 2) Protein: restriction of protein in diet to lower blood ■

cases to correct hyperaldosteronism. 2.

Fluid:

Salt & fluid restriction in severe oliguria, oedema & hypertension Fluid restriction: volume of urine/day + 500 ml insensible loss + 500 ml/r C fever.

Loop (furosemide) or thiazide diuretics with K supplements.

■

Dietary management for uremia & symptomatic treatment:

1. Fluid: 0

Complete bed rest.

2) Hypertensive encephalopathy: parenteral antihypertensive + cerebral dehydrating measures.

Iron, calcium & vitamin D supplement in case of

deficiency. in. Decrease glomenilar ieaka glomerulonephritis: poor prognosis 200 mg/D) —»these patients may maintain a stable GFR for decades. 17

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

N.B.:

❖ Nephrotic syndrome

❖ Nephritic syndrome

1. Onset

o

2. Process

0 Non inflammatory process

3. Proteinuria

o > 3.5gm/1.73m^/day,

o < 3.5gm/1.73m^/day

0 Selective & non-selective proteinuria

Gradual

o

Acute

o Inflammatory process

4. Albumin

0 m

0 Non-selective proteinuria o Normal or slightly J,

5. Edema

0 Peri-orbital edema then generalized

o

Peri-orbital edema

T

edema

6. Blood pressure

0

Normal

0

7. Intravascular volume

o

Variable

o t hypervolemia o i o Oliguria & azotemia

8. GFR 9. Urine volume 10. Hematuria

o

±

o

++-1-

11. Hypercholesterolemia 12. Hypercoagulable state

0

+

0

-

13. Urine 14. Cast

0 Frothy 0 Fatty cast

15. Diagnosis

0 Renal biopsy with immunohistochemistry and electron microscopy of the

16. Treatment

0

o Smoky/dark o

RBC cast

biopsy is the golden standard for diagnosis

0 Of the cause e.g. anti-streptococcal

Corticosteroids

0 Cyclophosphamide

& antihypertensive drugs

18

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Tubular And Interstitial Diseases

Inliynoo ttdfiey

1. Acute tubular necrosis : see acute RF

2. Cystic kidney diseases ; see hereditary renal diseases 3. Isolated tubular function defects

4. Interstitial nephritis

Tubulo-Interstitial nephropathy:Interstitial nephritis o Disorders affecting mainly renal interslitium & tubules o The glomeruli are spared in acute cases but are fibrosed in chronic cases. Chronic interstitial nephritis Acute interstitial nephritis Etiology :

1. Idiopathic

1. Idiopathic

2. Infection:

2. Infections:

o o o o

o Chronic pyelonephritis o Renal tuberculosis, Brucellosis, Diphtheria

Acute pyelonephritis Bacterial (streptococcal) Spirocetal (syphilis) Viral(EBV)

o IMN,CMV

3. latrogenic : hypersensitivity (Allergic) reaction to drugs e.g.: o Analgesic nephropathy: NSAID o Allopurinol o

Penicillin & sulfonamides

o o 4. o o

Cephalosporins Diuretics (furosemide & thiazides) Immunologic: SLE, Good pasture syndrome, Transplant rejection

3. o o o

latrogenic : Drugs: Analgesic nephropathy: NSAIDs Other drugs: Lithium, Cisplastin Heavy metals : Lead & mercury

Valve mmaint

4. Irradiation

5. Immunologic: SLE, Good pasture syndrome, transplant rejection 6. Reflux nephropathy (vesicoureteric reflux): o During micturition, urine may pass upward to ureters up to renal pelvis. 7. Metabolic: DM,Gout 8. Malignancy : multiple myeloma 9. Miscellaneous: obstructive uropathy. ❖ Pathology

0 Interstitial fibrosis, mononuclear cells infiltration, and 0 There is interstitial edema with heavy infiltrate by tubular atrophy. neutrophils, eosinophils and monocytes ❖ Clinical picture:

a

1. Tubular abnormalities : e.g. 1. Allergic: fever, arthralgia, skin rash 2. Infective interstitial nephritis = acute pyelonephritis o PCT defect: Fanconi syndrome o DCT defect: Renal tubular acidosis(RTA 4) o Medullary defect: polyuria & salt losing nephropathy 2. Endocrine deficiencies :anemia, renal osteodystrophies 3. Clinical picture of the cause & complication e.g. CRF Investigations Urine analysis : 1. Urine analysis: hematuria, proteinuria, WBCs casts Esinophiluria, hematuria, proteinuria, WBCs casts 2. Blood investigations: anemia Blood investigation : CBC: Esinophilia 3. Renal biopsy: interstitial fibrosis, mononuclear cells Renal biopsy : interstitial edema + intense infiltration infiltration, and tubular atrophy. by esinophils and variable acute tubular necrosis 4. Other investigations of CUE. Other investigations of ARE.

3. Clinical picture of the cause 4. Clinical picture of the complication e.g. ARE

1. o 2. 3. 4.

❖ Treatment 1. Treatment of cause:

0

o Stop drug & antimicrobial for infection

0 Treatment of complication: CRF.

Treatment of cause

2. Steroids: Prednisone 60 mg/day enhance recovery 3. Treatment of complication: ARE 19

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Infection of the kidney: Pyelonephritis(PN) Pyelonephritis

❖ Definition: inflammation of renal pelvis (pyelitis) and renal parenchyma (nephritis) usually bilateral except In unilateral obstruction.

Acute P\eloncphritis

Chronic Pyelonephritis (Reflux nephropathy) Etiology :

Infection:

Organisms: The commonest: E. Coll(85%) B. Staphyloeoceal, klehsiella C. Pseudomonas, proteus

Recurrent pyelonephritis due to Inadequate treatment of acute pyelonephritis mainly due to : Vesicoureteric reflux or obstructive uropathy Bad general condition or suppressed immune system. Resistant organism e.g. TB

1.

A.

tl

2. Mechanisms of infection :3 routes

Ascending Infection: retrograde spread of infection only in the presence of vesicoureteric reflux. B. Hematogenous:form septic focus C. Lymphatic : periureteric lymphatics from bladder or A.

Persistence of infection source.

Inadequate dose of antibiotics or bad choice of antibiotics

Presence of FB in the urinary tract.

gut 3. A.

B. C.

Predisposing factors: Stasis: due to recumbency, stricture, stones, prostate Metabolic factors : DM,gout, nephrocalcinosis Mechanical factors introducing infection : catheter, coitus (honey-moon cystitis)

uUMrwMl nftw

N.B.: 1. Females affected > males due to: o

Short urethra

o

Close to fecal contamination

o o

2.

luneO^fOno vextcAweMril

Absence of prostatic bactericidal fluid Pregnancy; hormones —> relaxation of ureters + compression by uterus ^ stasis Males > 40 are more common: due to big prostate

lunclApo aacuu-l#!(omkUms

B*cutri«t

*nO

aKendmg iixhKXion EnltMocoKcxi*

Pathology :

Gross picture: congestion and edema of the renal pelvis, and the cut section of the kidney may show abscesses or streaks of pus Microscopic: there is infiltration of renal parenchyma by Polymorphonuclear leukocytes (PMNLs)

Gross picture: the lesion is asymmetrical, with

shrunken scarred kidney & adherent capsule. Cut section can not differentiate cortex from medulla

Microscopic; Periglomerular fibrosis, atrophic tubules, interstitial infiltration by chronic inflammatory cells and extensive fibrosis

❖ Clinical picture: Acute pyelitis: Minimal symptoms with tender renal angles. 2. Acute pyelonephritis : A. Symptoms Constitutional manifestations: shivering, fever, o headache, anorexia, malaise. Pain in the loin radiating to the groin o Dysuria, frequency, turbid urine and hematuria. o B. Signs: Fever, tachycardia o Tendemess in renal angle o 3. Acute necrotlzing paplllltis: (acute papillary necrosis): In elderly diabetic. o In addition, there is hematuria due to o necrosis of renal papillae, renal colic & acute RF IVP show loss of part of one or more papillae o ❖ N.B.: Asymptomatic bacterluria: Organism > 100.000 ml -I- no symptoms o Occurs in pregnant females. o 40% develop pyelonephritis and it should be treated o 1. o

1. Asymptotic hematuria or proteinuria 2. Anemia

3. Secondary hypertension 4. Recurrent attacks of acute pyelonephritis 5. CRF

20

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Investigation :

1) Urine analysis: Volume : normal

o

Aspect; turbid Specific gravity : high pH: acidic with E. coli infection and alkaline with proteus infection Proteinuria, hematuria may be present

o o o

o

Microscopic examination :

o

Pus cells ; fft

Hyaline & white cells casts RBCs & organisms. 2) Urine culture & sensitivity from mid-stream urine: o Counts < 10,000 : contamination o Counts between 10,000 -100,000 : repeated o Counts > 100, 000 organism /ml: infection 3) Biood investigations: o t ESR, t PMNL. o Possibly bacteremia detected by culture. Anemia in chronic PN.

o

4) Renal function test o Acute PN: normal unless complicated by acute

o Chronic PN: impaired with affection of tubular

function more than glomerular function

renal failure

5) Renal imaging :PUT, US,CT, MRI,intravenous pyelography(IVP) o For predisposing factors e.g. stones o In chronic PN: IVP shows shrunken scarred & asymmetrical kidneys 6) Renal biopsy in chronic cases only to show the pathology.

7) Investigation for the causes ❖ Treatment;

I. Prevention (for recurrent UTI): Correction of predisposing factors: e.g. control of DM,removal of renal stones Avoid unnecessary nephrotoxic drugs Avoid undue catheterization

1.

Prevention:

A. Correction of predisposing factors: e.g. control of DM, removal of renal stones

B. Avoid unnecessary nephrotoxic drugs C. Avoid undue catheterization

Excessive fluids intake with regular emptying of

II.

Curative:

bladder.

1. Analgesics & antipyretics

To prevent honeymoon cystitis : Nitrofurantoin 100 mg at bedtime for 7 days

2. Antibiotics:

o Should be chosen according to culture & sensitivity o The doses should be reduced due to impaired Curative: II. excretion of the drug 1. Analgesics & antipyretics o The antibiotics should be used for long period of time. 2. Antibiotics: 3. Nephrectomy: A. Empirical antibiotic therapy (before the appearance o If the affected kidney is severely damaged to avoid the of culture and sensitivity results): damage of the other kidney. o Ciprofloxacin 500 mg /12hr for 7 days o Co - trimoxazole (trimethoprim/sulphamethoxazole): 2 4. Treatment of the cause & complication e.g. CRF & hypertension. tab (160/800mg)/12hr for 14 days B.

o o

3. o o

4.

Resistant cases: treated according to culture & sensitivity results: For E.coli: Ciprofloxacin 500 mg /12hr

For Pseudomonas: Carbenicillin 1 gm/ 6 hrs IV Change of the urine pH: If acidic give bicarbonate(NaHCOs)

If alkaline give ammonium chloride(NH4CI) Treatment of the cause & complication.

21

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Renal Vascular diseases

1) Arteries: thrombosis or embolization leading to renal infarction. 2) Arteriolcs and mici o\ asculature:

A. B. C. D.

Hypertensive nephrosclerosis leading to chronic renal failure. Malignant hypertension leading to acute or rapidly progressive renal failure. Scleroderma leads to malignant crises with hypertension and rapidly progressive renal failure. HUS / TTP —> renal failure, thrombocytopenia and hemolytic anemia.

E. Atheroeinbolic renal disease: ■

It is due to showers of choiesteroi rich atheromatous material from

■

ulcerated atheromatous plaques which may reach the kidney from aorta and / or renal arteries after catheterization of abdominal aorta or at renal artery angioplasty. Anticoagulants and thrombolytic agents may also precipitate cholesterol embolization.

■

It presents as rapidly progressive renal failure with systemic embolization (poor prognosis).

3) Renal vein thrombosis:

o It occurs in nephrotic syndrome, renal cell carcinoma and with thrombophilia. o It leads to acute renal failure, proteinuria and pulmonary embolism.

ReMi

artery

4) Renal Artery Stenosis (R.A.S.):

❖ ❖ A. B. o o

Flbromuscular Incidence: 1-2 % of all cases of hypertension dysplasla Atherosclerosis Etiology : Congenital: flbromuscular dysplasia, 25% of cases, usually women < 45 yrs. Acquired: Atherosclerotic type, 75% of cases, usually males > 60 yrs. Others: dissecting aortic aneurysm, PAN,renal artery thrombosis, embolism, trauma & neurofibromatosis .

Pathogenesis:

G

Angiotensinogen

Renin

RAS —> renal ischemia

Healthy kidney

v Angiotensinogen I

XI Angiotensinogen II

l-O-

G

Aldosterone ^ sodium & water retention —> HTN

o

r

VC^ HTN

J'=^

Ischemia stimulates JGA to release Renin

o Renin hydrolyzes circulating, locally produced angiotensinogen to angiotensin I o Pulmonary ACE hydrolyzes Angiotensin 1 to Angiotensin II

o All acts on vascular, glomerular, adrenal & other receptors —> systemic VC , Na^ & H2O retention o All is deactivated by hydrolysis to Angiotensin III and IV o In early phases contralateral kidney antagonizes excess Angiotensin II. o Later HTN ensues followed by renal impairment with nephrosclerosis of this contralateral kidney,

o At this stage nephrectomy ofthe originally diseased kidney with RAS may be of no value. 22

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

❖ Clinical picture & criteria to suspect RAS: 1. Renovascular Hypertension (R.A.S.)o Sudden onset < 30y or > 50 years old o

Short duration

o o 2. o

Recent worsening of HTN Refractory HTN Kidney: Deterioration of kidney function with ACEI or ARBs

o Unexplained hypokalemia with metabolic alkalosis o Unexplained progression of renal failure 3. Abdominal bruit

4. ❖ 1. 2.

Abdominal US: asymmetrical size of the kidney Investigations: Biochemical: Hypokalemia with metabolic alkalosis Rapid sequence IVP(Old test), the affected side shows:

Renal .Artery Straosis

o Smaller size kidney compared to other one

o Delayed appearance & delayed clearance ofthe dye 3. US: the ischemic kidney may be smaller. 4. Renal isotopic scan:

o Showing defect in perfusion o In unilateral cases there is fall in uptake on the affected side following ACE inhibitor administration.

5. Renal angiography (gold standard in diagnosis) show stenosis in the affected artery. 6. Renal Duplex scan.

7. Renal vein renin: It is f in the ischemic side.

8. Magnetic resonance angiography: in patients with renal impairment to avoid contrast nephropathy. Treatment:

o

Medical Antihypertensive to J, RAAS: ACEI or PB(ACE inhibitors are contraindicated in bilateral renal artery stenosis^ ARE)

2.

Surgical:

1.

❖ Indications: o

Refractory HTN,intolerance to ACEI, deterioration of kidney function i.e. > 30% increase in creatinine

o

Intrarenal resistance index < 80 % Methods: guide wire with balloon

artery

T

catheter

atheroma (fatty plaque) Healthy kidney

guide wire

-balloon

Diseased

kidney is removed

A. Percutaneous transluminal renal angioplasty: balloon dilation with stenting of stenosed artery B. Surgical revascularization (arterial reconstmction) C. Surgical removal(nephrectomy): in refractory HTN

23

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Acute Renal Failure Bfood HOW

SJtoreguIation

Renal Arten

t/Vf.

f

j

Glomerulus

(Inuagwmerular| pressure matntainec)

Ufirte.iFfow

Ureler

Anerenl

Efferent

arteriole

arterwie m

Bladder

Prostate (in Men) ^ Tubule

Urethra

ISCHEMIC

fQ

TOXIC

ATN

Decreased

glomerular filtration

Afferent

J

arteriolar cxjnstriction

Loop of Henle

Ischemic/toxic itsu"

LQOp of Henle Back-

V* Oliguria : o

Acute Uremia

o o

Hypervolemia & Hyperventilation (Kussmaui's Breathing) Hyperkalemia,Hyperphosphatemia, Hvpocalcemia.

V

-

u

Obstrucbon

Chronic Renal Failure

int^aglomptviaf

Damaged endothelium

> Sclerosis Protemuria

1 Ca absorption

tPTH

t Serum P 1 Vitamin D activation

Impaired Renal Function

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Tubuar

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Pre-Renal Failure : J,), blood supply to kidneys; renal hypoperfusion:

Endogenous:

Sepsis: gram -ve septicemia, septic abortion Heavy metals: mercury, lead, radioeontrast agents Drugs: Aminoglycosides, ACEI, NSAIDS, Amphotericin B.

Exogenous:

Hemolytic crisis, rhabdomyolysis, multiple myeloma Hypemricemia, Hypercalcemia

111. Post Renal Causes : urine retention backpressure —> anuria. 1. Bilateral urethral, bladder or ureteric obstmction e.g. stones or tumors 2. Unilateral ureteric obstruction if single kidney. 3. Urethral obstruction:cancer, stone, prostate

o Vasculitis e.g. SLE,PAN,HSP & sclerodermic crisis o Microangiopathic hemolytic anemia e.g. malignant HTN,HUS,TTP & DIG.

4. Vascular diseases:

■ ■ ■

ii.

i.

25

DM : diabetic nephropathy: the most common cause Enzymatic defect as in lipid storage disease Fe: hemolytic anemia D.

8.

disease e.g. elinical signs & symptoms

Azotemia : biochemical changes e.g. t blood Urea and BUN ratio Uremia or Uremic syndrome = Azotemia + systemic manifestation of renal

N.B.: in AKI or CRD:

Mechanical obstruction (obstructive uropathy): bilateral ureteric obstruction, urethral obstruction e.g. prostate enlargement.

G. Gout

F.

E.

1 Ca"^^: nephrocalcinosis

Amyloidosis Plasma cell myeloma (Multiple myeloma)

C.

B.

A.

7. Metabolic:

B. Toxic ATN (toxic nephropathy) due to:

Hemolytic uremic syndrome(HUS) Thrombotic thrombocytopenic purpura(TTP)

Hypertensive nephrosclerosis (atherosclerosis): 2"'' common cause.

o

o

Vasculitis e.g. SLE,PAN,HSP & scleroderma Microangiopathic hemolytic anemia(MAHA):

b.

a.

6. Vascular:

o

■ ■

Polycystic kidney, hypoplastic kidney. Fanconi syndrome. Renal tubular acidosis Infection : chronic interstitial nephritis, chronic pyelonephritis, renal TB latrogenic; drugs: Analgesic nephropathy e.g. NSAlDs Antibiotics e.g. Aminoglycosides & Tetracycline Heavy metals e.g. lead poisoning

5. Irradiation

o

o

o

4.

3.

o

o

o All causes of prerenal failure if prolonged

3. Tubular: acute tubular necrosis(ATN)which is either: A. Ischemic ATN (vasomotor nephropathy):

2. Interstitial: acute interstitial nephritis due to infection (acute necrotizing papillitis) or drug allergy(NSAID, antibiotics e.g. penicillin).

11. Renal Causes, intrinsic parenchymal renal disease : 1. Glomerular lesion: all causes of acute GN especially RPGN & membranoproliferative GN

3. Hepato-renal syndrome.

dissection

C. Obstructive shock:massive pulmonary embolism D. Cardiogenic shock e.g. HF, Acute MI 2. Bilateral renal artery occlusion: bilateral thrombosis or embolism & aortic

Idiopathic: chronic glomemlonephritis e.g. rapidly progressive GN

2. Inherited :

1.

❖ Etiology

B. Hypovolemic shock e.g. Blood loss, Bums,Polyuria, severe diarrhea or vomiting

A. Septic shock

1. Shock:

I.

reversible.

Chronic Renal Failure (Chronic kidney disease = CKD) o Gradual (over months or years) deterioration of renal function (Glomerular & Tubular) with disturbance of body homeostasis. This is usually irreversible.

❖ Definition

AKI is a clinical syndrome characterized by rapid (over hours or days) deterioration of renal funetion (Glomerular & Tubular) causing oliguria up to anuria and can be

Acute Renal Failure (Acute Kidney Injury = AKl)

Renal failure

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

1.

renal perfusion:

Urinary Na X Plasma Creatinine

Plasma Na X Urinary Creatinine

Na clearance

FeNa can be calculated from: -

Creatinine clearance

High osmolality > 500 mosmol/L, High specific gravity > 1020 Low urinary Na < 20(mEq/L)& fractional excretion of sodium (FeNa)< 1%

urine is concentrated with :

0

Extent of loss

Tubules & basement membrane

(PCT & DCT)

Patch necrosis of whole tubules

A. Ischemic ATN

Tubular cells only Better prognosis

tubules

Diffuse necrosis of proximal

o

Post-Renal ARF:

Obstruction —> t back pressure in renal tubules —> ], pressure gradient for glomerular filtration —> J. GFR.

III.

26

B. Urine is diluted: Osmolality 40 mEq/L & fractional excretion of sodium >1%.

< 40 : I(N.B. Urea = 2 BUN)

A. Plasma : creatinine clearance affected more than urea clearance so urea : creatinine ratio

In ATN the lesion in renal tubules:

urine volume & kidney function to normal(may take 3-6 m).

Retention of urea —+ osmotic diuresis

Recovery (convalescent) phase: complete recovery of tubules —> Gradual return of

o

Polyuric (diuretic) phase (for 2- 4 days): Relief of tubular obstruction due to partial recovery of tubules Recovery of glomeruli before distal tubules —> f GFR without tubular reabsorption diuresis with dehydration, hypotension & electrolyte loss e.g. hypokalemia, hyponatremia

3.

o

o

2.

D. Glomerular contraction —> I surface area for fdtration.

C.

B.

0 0

0

B. Toxic ATN

Obstruction of tubules by debris shed from ischemic tubular epithelial cells Back leak of glomerular filtration in proximal tubule due to loss of function of tubular cells Reflex VC of afferent arteriole due to "f renin (vasomotor nephropathy)

Prognosis 0 Bad prognosis Oiiguria occurs secondary to:

0

Affected site

A.

o

100

Renal tubular function is intact in pre-renal states —Na and H2O reahsorption —> so

A. Urea:

0

contributes to creatinine clearance)

Nausea, vomiting, Uremic frost, pruritus.

breath

0

0 Pale urine.

pigments Earthy looking skin

B. Urochrome

Polvuria in stage 1:

0

0

2)

Associated metabolic acidosis

Hyperkalemia: With oiiguria

hypernatremia & hypervolemia

excretion —+ Na & H2O retention —»

activity —> failed Na & H2O glomemlar

Salt retaining nephropathy : Glomerular lesions with high rennin

Osmotic diuresis due to retention of urea & waste products. Failure of tubules to respond to ADH.

Oiiguria: Further ], GFR in late cases due to complete destruction of all nephrons Calcium & Phosphate {P04'^ (P)} : In CRF there's | P & J,, normal, | Ca^ due to: "[■ P due to failure of glomerular excretion ^ | intestinal excretion of P —> binds Ca & prevent its absorption ^ metastatic calcification & deposition in muscles, heart & joints. J, Renal production of one alpha hydroxylase —> J. vitamin D activation intestinal absorption of Ca^^ —> Ca'^ level. t P , J. vitamin D & J. Ca" —> 2ry hyperparathyroidism with low or nonnal calcium level ^ prolonged cases transforms to 3ry hyperparathyroidism with high Ca'^ level 2) o

D.

c.

b.

The single nephron fdtration rate is increased in remaining glomeruli which overwhelm the tubular capacity to concentrate or dilute urine hence there is polyuria with a low fixed specific gravity at 1010 (isothenuria)

1) a.

Water balance:

1) Hypokalemia: 0 Secondary to hyponatremia —> f aldosterone —> t fecal loss of K

Potassium

reabsorption —> Na loss & polyuria — hyponatremia & hypovoiemia

Tubular lesions —>■ failed Na & H2O

C.

B.

o

1) Salt losing nephropathy :

A. Sodium :

2) o

Due to failure of excretion of H ions & Failure of formation of HCO3,

3. Disturbance in electrolyte balance:

o

2. Metabolic acidosis: Kussmaul's breathing(deep rapid breathing).

D. Uric acid: gout.

0 0

Uremic (ammoniacal)

C. Creatinine, methyl guanidine, guanidinosuccinic acid, indoles, aliphatic & aromatic amines, phenols,|32 microglobulin 0 Peripheral neuropathy, ataxia 0 Pericarditis, Bleeding, hemolysis

Manifestations of renal failure starts when GFR < 30 % of normal:

1. Retention of waste products:

❖

I & 2 leading to glomerular hyperfiltration ^ glomerular HTN (f intraglomemlar pressure) with proteinuria ^ "["I") glomerular damage —> glomerular fibrosis & sclerosis —>■ complete destruction of all nephrons ^ progressive iJ.i of renal function —> CKD

response to prolonged hypovoiemia and hypotension leading to : I urine flow —> J, GFR and .1 urea clearance ^ "f blood urea and urea : creatinine ratio > 40:1 (N.B. Urea clearance depends on glomerular filtration mainly while tubular secretion

ARF in hypovolemic patients). Reflex sympathetic activation, aldosterone & ADH (vasopressin) production in

2. Activation of RAAS^ efferent arterioiar constriction & systemic HTN

1. Compensatory hypertrophy of remaining nephrons with afferent arterioiar dilation

Pathophysiology ❖ Irreversible loss of nephrons due to the original disease —>• t cytokines leading to :

Reduction of RBF is compensated by renai auto-regulation to maintain GFR; Auto-regulation(MAP 80 - 180 mmHg), acts through PGs causing VD of afferent arteriole & Angiotensin II causing VC of efferent arteriole (i.e. NSAID & ACEI may precipitate

Pre-rcnal ARF

n. Renal ARF : established structural abnormalities in kidneys: The commonest cause is acute mbular necrosis(ATN): occurs in 3 phases : 1. Oliguric phase (2-4 weeks):

o

o

3.

2.

o

I.

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Uremic encephalopathy with Confusion, Convulsion & Coma: see later.

Clinical picture of the cause:

3. In post-renal cases e.g. renal colics with anuria & hematuria.

2. In renal causes e.g. history of nephrotoxic drug intake or sepsis.

collapsed neck veins, inelastic skin, sunken eyes.

27

1. Pre-renal cases e.g. evidence of dehydration & hypovolemia: low BP,rapid pulse,

ii.

o Gradual return of urine volume & kidney function to normal o Complete recovery may be delayed to a period of 3 - 6 months,

hyponatremia III. Recovery Stage;

II. Polyuric (Diuretic) stage : o I UOP > 2 L/day up to 10 L/day O Clinically : dehydration, hypotension & electrolyte loss e.g. hypokalemia,

A. Anemia with pallor due to impaired erythropoiesis, hemolysis, bleeding tendency. B. Hyperphosphatemia & hypocalcemia : see CRF . C. Impaired immune response —»infection

5. Features of:

D. Muscle : Asthenia^ weakness, hyporeflexia up to flaccid paralysis

C. GIT ; Atony^ anorexia, nausea, vomiting, constipation up to paralytic ileus

o Prolongation ofPR interval, Absent P wave. Wide QRS (Sine wave) o Bradycardia, VT/VF, asystole & arrest.

wave (camel hump shape = Himalaya T), others:

❖ EGG : Earliest change is prominent T wave = hyperacute T wave: tall peaked T

A. CNS: Apathy mental confusion, convulsion & coma B. CVS: Arrhythmia :

4. Features of hypcrkalemia:

3. Features of Metabolic acidosis: Kussmaul's breathing (deep rapid breathing).

pulmonary & brain edema with confiision, convulsion & coma.

2. Features of hypervolemia (fluid overload): o Hypertension, congested neck veins, congestive HF & Edema; LL edema,

o

o Asterixis, Anemia & Anorexia, nausea, vomiting & hiccough, o Pruritus, Peptic ulcer(GIT bleeding), Pericarditis, Pleurisy, Peritonitis

1. Features of acute uremia :

I.

Stroke e.g. ICH due to HTN crisis & bleeding tendency

hypertensive encephalopathy.

Uremic Coma due to: acidosis, electrolyte imbalance, hypervolemia &

Confusion, lack of Concentration, Convulsions

Slurred speech & inverted sleeping rhythm

Personality changes. Psychosis.

(hard) water to dissolve dialysate for dialysis. Uremic Encephalopathy: Global CNS dysfunction: Apathy, Agitation, Asterixis (flapping tremors)

Dialysis Dementia: due to aiuminium deposition in brain with using unpurified

vomiting, confusion & convulsions.

Hyporeninism: Diabetic nephropathy, obstructive uropathy, interstitial nephritis

N.B: CRF patient with normal or low BP: Salt losing nephropathy e.g. interstitial nephritis

Arrhythmia secondary to electrolyte imbalance, ischemia & HF. Heart Block due to Ca deposition in bundle of Hiss BP: Hypertension secondary to salt & H2O retention & RAAS activation Pericardium: Uremic dry pericarditis, hemorrhagic pericardial effusion with tamponade (especially with hemodialysis)& Constrictive pericarditis. Systolic and diastoiic dysfunctions : SD is due to myocardial fibrosis & calcification, J, camitine and selenium DD is due to LVH —»■ hypotension during hemodialysis.

Atherosclerosis secondary to hypertension & hyperlipidemia(J,clearance)j

CVS:

Band keratopathy on the cornea (due to Ca deposition) Red eyes (conjunctival VD due to metastatic Ca deposition) Retinopathy ± Retinal detachment.

Uremic Amaurosis(sudden loss of vision due to retinal artery spasm)'

Ocular:

J, Na: Muscle cramps + restless leg syndrome.

J, Ca: Muscle twitches, tremors & but tetany is rare (acidosis >1 Ca ionization)

10. Muscle :

9.

7.

Caused by rapid reduction of plasma urea level by dialysis ^ J, plasma osmolarity —> H20 shift intracellularly brain edema —> headache, nausea,

Disequilibrium syndrome:

Autonomic neuropathy e.g. Orthostatic hypotension, gastroparesis & impotence. Peripheral neuropathy & myopathy due to renal osteodystrophy. Carpal tunnel syndrome(due to (32 - microglobulin —» amyloid deposition).

Ataxia due to toxic effect on labyrinth.

Headache: Dull (toxic VD), Throbbing(HTN), t ICT(HTN encephalopathy)

CNS:

❖ Clinical picture

i. Clinical picture of ARF,it passes into 3 stages: Oligiiric(UOP < 400 ml/day) or Ainiric Stage(No UOP forl2-24hr):l-6 week

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Bone: Renal osteodystrophy:

Joints

Gout: t uric acid

o Pseudo-gout(deposition of Ca pyrophosphate).

o

11.

o Rugger Jersey spine : alternating sclerotic & porotic bands in the vertebra

3. Osteosclerosis due to direct effect of excess PTH:

A. Osteoporosis e.g. salt & pepper in skull X-ray B. Osteitis fibrosa cystica(Brown tumor = von Recklinghausen's disease of bone): replacement of bone by fibrous tissue with cyst formation C. Long standing secondary hyperparathyroidism will lead to adenomatous transformation in parts ofthe hyperplastic parathyroid glands —> tertiary hyperparathyroidism —> f Ca level

aluminium in dialysate fluid) 2. Effect of secondary hvpemarathvroidism: t oseoclastic activity :

o i Vitamin D ^ i intestinal absorption of calcium o Accumulation of aluminium in bone (phosphorus binders containing

Pallor: due to anemia.

12)Hematological system

Normocytic normochromic: Anemia of chronic illness: J, HP hormone release by the renal interstitium. Uremie hemolysis (J, life span of RBCs) Macrocytic: Loss of folic acid in dialysis.

J, Platelets adhesion due to J, VIIEVon Willebrand factor release from endothelium

28

13)Complication of hemodialysis e.g. Air embolism. Bleeding, B,C Hepatitis, 14)Complication of peritoneal diah sis e.g. Abdominal hernia, Peritonitis, organ injury

C. J, Chemotaxis of WBCs.

o I Platelets aggregation as guanidosuccinic acid ^ i platelet activation by ADP III. WBCs: t liability for infection (uremie toxins, acidosis, malnutrition)(Me to: A. i Antibody response of lymphocytes & lymphocytopenia B. I Phagocytic activity of polymorphonuclear leukocyte.

o

❖ N.B.: CRF patient with no or mild anemia(|EP hormone release): o Polycystic kidney, Hypemephroma with CKD & Hydronephrosis. H. Platelets: Thrombasthenia —> bleeding tendency:

B. o o C.

o Bleeding: due to GIT ulcers, purpura, and repeated dialysis.

I. RBCs(anemia): A. Microcytic hypochronic:

o Uremie frost(whitish power on skin due to deposition of urea crystals after evaporation of urea through sweat)

albicans, skin Dryness

o t PTH (direct action on skin), Ca deposition, Candida

3. Pruritis: due to

2. Purpura (bleeding tendency ), Pitting LL edema (frenin)

o

o Brown & Yellow: melanin & urochromogen deposition

due to mixture of three colors:

10)Skin

1. Pigmentation: Earthy look(uremie facies)^

1. t Insulin level (J, insulinase activity) ^ I insulin requirements in diabetic patients. 2. t PTH: Hyperparathyroidism. 3. t Prolactin & luteinizing hormone : Infertility & gynecomastia in males or menstrual dysfunction in females 4. J, Testosterone level —»impotence & 4 spermatogenesis. 5. i Vitamin D activation (J. 1 a hydroxylase activity). 6. I Erythropoietin ^ anemia. 7. J. GH secretion in children —»• stunted growth.

11)Musculoskeletal system

for masses ^ UT obstruction.

Bladder & prostate: should be examined

GN.

Shrunken: Chronic pyelonephritis or

1. Osteomalacia due to:

I.

2.

#

Enlarged in amyloidosis, polycystic kidney, bilateral hydronephrosis,DM.

Kidney:

8) Urinary symptoms

7) Pancreas: Pancreatitis (especially with hyperparathyroidism).

6) Liver: Hepatitis B & C in patients on dialysis & blood transfusion.

4) Stomach: Anorexia, nausea, vomiting, peptic ulcer(f gastrin) and delayed gastric emptying, hematemesis 5) Intestine: Constipation (uremie neuropathy + hyperparathyroidism) or uremie dysentery.

1) Mouth: uremie breath (ammoniacal fetor), coated tongue, bitter taste, stomatitis, ulcers (urea excreted in saliva with splitting by bacteria into ammonia) 2) Esophagus: Candidiasis & reflux oesophagitis. 3) Diaphragm : hiccough (central effect)

9) Endocrinal system

2) Uremie Asthma: bronchial irritation hy urea —+ bronchitis & bronchospasm. 3) Pulmonary infection 4) Pulmonary edema(f Renin —capillary permeability) 5) Pleurisy & Pleural effusion.

(deep rapid breathing)

1) Acidotic breathing : Kussmaul's breathing

4) Chest

Continue clinical picture of CHRONIC renal failure: 5) GIT

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Injury

Failure

2

3

7.

6.

5.

4.

o

o

o

i 50% i 75 %

i 25 %

anuria for 12 hours

90

❖ GFR (ml/min)

(if $ X 0.85 to compensate for smaller muscle mass

❖ Description Kidney damage with normal or f GFR Kidney damage with mild (.GFR Moderate J.GFR Severe j,GFR Renal failure (End stage renal disease)

72 X serum Creatinine

(140-Age)X BW in kg

❖ Investigation 1. Urine analysis:

5

4

3

2

1

❖ Stage

Equation:

Creatinine clearance : it equals GFR; Normal value : 100-120 ml/min

ATN.

t: K,P i : HC03, pH, Ca, Na

Normocytic normochromic anemia

Blood investigations:

Pigment casts: hemoglobinuria (hemolysis) or myoglobinuria (crush syndrome).

White cell casts & pus cells ^ acute pyelonephritis.

Epithelial casts

1. Plasma : BUN : creatinine ratio

Sodium mEq/L

3. Fractional excretion of sodium

o

o Specific gravity 0 Osmolality mosmol/L

2. Urine :

>40 > 1%

1020

Intrinsic renal e.g. ATN 20 : 1

f: Urea, creatinine, uric acid I: GFR,creatinine clearance. FeNa: < 1 % in pre renal, > 1% in ATN Renal imaging e.g. PUT,US, CT: in post renal causes to detect obstruction Renal biopsy: if no recovery > 1 month, unclear cause, diagnosis rather than ATN ECG: see features of hyperkalemia. Investigation of the cause & complications.

29

Broad cell casts due to dilated renal tubules (characteristic) Blood investigations: Anemia(3 types; see before), J, Lymphocytes, thrombocytopenia | & bleeding time, i or f: Na, K, Ca (N.B.: Ca may be J, or normal or even f) t: P i : HC03, pH

■ 2. o o o o o

o Salt & pepper in skull o Osteitis fibrosa cystica o Rugger Jersey spine

8. Investigation for the complications e.g. skeletal survey for hyperparathyroidism; Bone: X-ray may show:

6. ECG: see features of hyperkalemia. 7. Investigation of the cause.

o Shrunken kidney: chronic GN or chronic pyelonephritis o Large kidney: Amyloidosis, Polycystic kidney, bilateral Hydronephrosis, DM. 5. Renal biopsy: for the pathology

o 1: Urea, creatinine, uric acid o |: GFR,creatinine clearance. 4. Renal imaging e.g. PUT, US,CT:

3. Renal function test:

Microscopic examination : Granular cell casts

■

Microscopic examination :In renal causes: Red cell casts ^ Acute GN.

o

o Proteins : mild proteinuria

Proteins : mild proteinuria

o Volume: early stages polyuria (2-4 L/day), late stages oliguria Volume: oliguria / anuria in early stages, polyuria in diuretic phase o Aspect: Pale urine Aspect: dark; hematuria in renal and post renal causes. Specific gravity: in prerenal > 1020, in ATN low fixed at range of 1010 (isothenuria) o Specific gravity : low fixed specific gravity at range of 1010(isothenuria)

Urine analysis:

ESRD

3. Renal function test:

o

o

o

2.

Risk

1

< 0.5ml/kg/hr for 6 hours < 0.5ml/kg/hr for 12 hours < 0.3ml/kg/hr for 24 hours or

UOP

Persistent ARF= complete loss for renal function > 1 month End stage renal disease > 3 month.

t >1.5 X baseline t > 2 X baseline 1 > 3 X baseline

Loss

Serum creatinine

Criteria

Staging

GFR

Staging

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Treatment Of cause (before persistent oliguria):

o In polyiuia: give amounts sufficient to produce 2-3 L urine /day. o In oliguria: fluid restriction: voltune of urine/day -t- 500 ml insensible loss -I- 500

❖ Treatment

In oHguric phase:

Treatment of established ARF:

Dialysis if K > 7 mEq/L.

Urea > 200 mg/dl

o

30

UOP < 200ml/12hr

o

o

GFR < 10 ml/min.

o HC03< 10 mEq/L

o Creatinine > 10 mg/dl. In Polyuric(d uretic) stage:

pH 7 mEq/L.

o Give IV fluids & correction of electrolytes e.g. K & Na supplements, ill. In recovery phase: gradual)in protein intake. III. Treatment of complications e.g. HF

11.

o

o

b. Laboratory:

a. Clinically :fluid overload e.g. CHF, APE, pericarditis & uremic encephalopathy.

6. Dialysis: indications:

o Dietary restrietion e.g. milk + give antacids A10H3 or CaC03 to J, P absorption.

5. Correction of hyperphosphatemia:

D. Antagonize effect on heart:Ca glueonate 10%, 10 ml solution slowly. 4. Treatment of metabolic acidosis: IV NaHC03 if HC03 < 10 mEq/L or pH Causes

metabolic acidosis with NAG

0

vitamin D : for rickets

supplement.

0 Sodium bicarbonate, K

36

Sodium bicarbonate

Plasma Bicarbonate : 10-15 mEq/L Initially urine pH > 5.5 then

0 K supplement

0

❖ Treatmen t:

o Plasma Bicarbonate : < 10 mEq/L o Urine pH> 5.5

metabolic acidosis with NAG

❖ Investigation: Hypokalemia & hyperchloremic o Hypokalemia & hyperchloremic

Bone: rickets or osteomalacia

❖ Clinical picture: Acidosis ^ I tubular reabsorption of Ca^^ o Anorexia, nausea, vomiting hypercalcuria stones & o Children: retardation of growth nephrocalcinosis

A. Amphotericin B, expired tetracycline, excess Vitamin D B. Post renal transplantation C. Cancer: multiple myeloma

2. Secondary: o Post renal transplantation o Fanconi syndrome

1. Primary; inherited (congenial)

❖ N.B. Type III RTA: is a mixture of type I & II.

Failure of DCT to secrete

1. Primary: inherited (congenial) 2. Secondary:

o

|

❖ Definition

Type I RTA (Distal)

resistance to its action.

Aldosterone deficiency or

Type IV RTA

Chronic tubulointerstitial disease

Sodium bicarbonate

0 Anti-hyperkalemic measures

0

o Plasma Bicarbonate: 15-20 niEq/L

acidosis with NAG.

o Hyperkalemia & mild hyperchloremic metabolic

o Clinical picture of the cause

common)

o Diabetic nephropathy (the most

o

❖ Hyporeninemic hypoaldosteronism: 1. Primary: Addison's disease, congenital adrenal hyperplasia 2. Secondary: o Adrenal insufficiency o Potassium sparing diuretic

o

- ^

Drugs & The Kidney

❖ Drugs causing renal impairment: 1. o o

2.

Pre-rcnal impairment: Hypovolemia: diuretics Decreased cardiac output: Beta-blockers Renal damage:

o

Glomerular lesion: D-penicillamine

o

Interstitial nephritis: NSAID, Penicillin, Cephalosporin, and thiazides Diuretics

3.

Acute tubular necrosis: Aminoglycosides, ACEI, NSAIDS, Amphotericin B,contrast nephropathy. Post renal obstruction: Retroperitoneal fibrosis induced by long use of methysergide

4.

Nephrogenic diabetes ineipidus: Lithium, Doxycycline, methoxyflurane

o

>> Drug precautions in patients with renal impairment: j 1. i insulin catabolism dose of insulin in DM (decreased) 2. I elimination of water soluble drugs e.g. Gentamycin 3. i protein bound drug | e.g. dose of phenytoin

4. Drugs associated with increased catabolism will increase urea level e.g. corticosteroids and tetracycline. Renal Involvement In Systematic Diseases

1. Renal involvement in DM,liver disease, systemic vasculitis, SLE, and rheumatic disorders

2. Infection related glomerulopathies , Dysproteinemias and Amyloidosis. Renal Involvement in internal malignancy: Glomerulonephritis e.g. membranous GN.

For more details see related chapter for each one

Urinary tract obstruction leads to acute or chronic renal failure Vascular .

Hemorrhagic cystitis of cyclophosphamide.

Tumor lysis syndrome: acute uric acid nephropathy and renal failure may occur

Interstitial

after lysis of tumor cells sensitive to chemotherapy or radiation Normal parameters:

Plasma

Wall " ECF

Plasma , \1embrane

Electrolyte & Acid -Base Imbalance

Fluid compartment: water balance due to balance between input controlled by thirst sensation and output controlled by renal ADH system

ICF

Total body water(TBW): 0.5 body weight in female, 0.6 BW in male. 1. Intracellular compartment: 60% of TBW i.e. 25 L

2. Extracellular compartment 40% of TBW e.g. 17 L A. Interstitial: 32% of TBW i.e.

B. Intravascular : 8 % of TBW i.e.

13.5 L

3.5 L

Abnormalities of water include: hypovolemia (see: pre-renal causes of ARE & shock)& hypervolemia N.B: Plasma osmolarity {osmolality} = 2Na^+

Blood glucosemg/dl , BUN mg/dl 18

.,w ,

,

— + ——^ = 280-290 mosmoFL {mosmol/Kg} 2.8

Normal biochemistrv values of:

. pH • PaCo2 . r •

HC03-

•

Cl"

• Ca^^

0

7.35-7.45

0 35- 45 mmHg 0 3.5-5.5 mEq/L

•

Na+

0 135-145 mEq/L

•

Albumin

0 3.5-5.5 g/dL

• Total protein

o 6-8gm/dl

o 22-26 mEq/L 0 95-105 mEq/L -^2

H-2

❖ Normal serum calcium level: 8.5 -10.5 mg/dl —^4-5 mEq/L —> 2-2.5 mmoFL H-2

-h2

1. Ionized (free & active): 50% i.e. 4- 5 mg/dl —>2-2.5 mEq/L —> 1 - 1.25 mmol/L 2. Non ionized (reserve): 50 %: 0 40 % bound to albumin & 10 % with anions e.g. Citrate 37

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

Hypernatremia

Hvponatreniiii

0 Serum Na< 135 mEq/L

I

|o Serum JNa> 145 mnq/L ❖ Causes:

Na gain (Tintake or i loss), or Water deficit(t loss or i intake) *:• N.B. hyperosmolarity is not always associated with

Water gain (tintake or i loss), or Na deficit(T loss or i intake)

❖ Causes of pseudo- hyponatremia (hyponatermia

hypernatremia e.g. marked hyperglycemia & ketoacidosis.

without hypo- osmolarity), causes : —> Check plasma osmolarity:

1. Normal osmolarity: Hyperlipidemia, hyperproteinemia 2. High osmolarity:

❖ Causes of hypematremia according to the volume status of the patient (extra-cellular volume): —> Check volume status:

0 HyperGlycemia, t Glycine. o Mannitol, Maltose.

5.5 mEq/L & ECG changes (see renal failure)

o

o Features of hyperkalemia o Chest: Kussmaul's breathing o CVS: myocardial depression , hypotension &(VFA^T)in severe cases o Confusion, Convulsion , Coma, Death

Addison's Disease

o RTA & Hypoaldosteronism e.g.

o Features of hyperkalmia (See before in RE)

Clinical picture

Addison's Disease

8. RTA IV & Hypoaldosteronism e.g.

7. Renal failure

o

(Hyperchloremic): Amino acid infusion in TPN,Blood transfusion (Old stored) o 1 Cl" containing fluids e.g. NaCl

B. Normal AG metabolic acidosis

Renal failure

Rhabdomyolysis

o

o

2. Bum,Blood Transfusion (Old stored). 3. Cellular damage: rhabdomyolysis, hemolysis, tumor lysis syndrome. 4. Dmgs: ACEI/ARBs, pB, Potassium sparing diuretics, Suxamethonium, heparin. 5. Digitalis Toxicity 6. DKA & insulin deficiency

1. Acidosis

A. o o o

mEq/L High AG metabolic acidosis: Lactic acidosis e.g. shock Ketoacidosis e.g. DKA Salicylic acid intoxication

❖ According to Anion Gap (AG): o {(Na^+K+)-(Cl- + HC03-)} = 10-12

Metabolic acidosis

clenching o Marked T WBCS & PLT B. True hyperkalemla:

o Traumatic venipuncture with repeated fist

Hyperkalemla > 5.5 niEq/L ❖ Etiology A. Pseudo hyperkalemla : o Hemolyzed sample

1 1

Kcspiratory aikalosis

Respiratory acidosis

❖ Etiology ❖ Causes of hyperventilation syndrome:

I. Causes of hypoventilation : 1) Obstruction hypoventilation: A. Upper airway: inhaled foreign body, laryngeal edema, spasm & tumor B. Lower airway: 0 Chronic obstructive pulmonary disease(COPD):

A. Central stimulation :

1. Anxiety 2. Pain, Pregnancy 3. Cancer

4, Drugs e.g. Acetylsalicylic acid & Aminophylline 5. Encephalitis & meningitis

Chronic bronchitis & Emphysema.

6. Fever

0

Bronchial Asthma & Asthmatic bronchitis

7. Gram -ve septicemia 8. Hysterical & Head injury 9.

B. 1.

2. 3. 4.

5. 6.

0 Bronchiectasis & Cystic fibrosis. 2) Restrictive hypoventilation : Ischemia & Stroke. i. Disorders of neuromuscular apparatus: Peripheral stimulation : 1) Depression of respiratory center as in head injury, Hypoxia & High altitude stroke, encephalitis, brain tumors, drugs (opiates). Heart failure, Hypotension 2) Interference in the impulse transmission to Hepatic failure & metabolic acidosis respiratory muscles: Anemia, Bronchial Asthma. 0 Lesions in spinal cord e.g. Transverse myelitis, spinal Pneumonia, Pulmonary Edema,Pulmonary Embolism. fracture, anterior spinal artery occlusion Interstitial lung fibrosis 0 Lesions anterior horn cells : poliomyelitis 0 Lesions in peripheral nerves e.g. Guillain-Barre syndrome 0 Lesion in neuromuscular junction: myasthenia gravis. 0 Lesions in respiratory muscles: myopathy. ii. Decreased compliance: A. Lung diseases e.g. Interstitial lung fibrosis B. Pleural diseases e.g. pleural fibrosis, tension pneumothorax, massive pleural effusion C. Chest wall diseases :

0 Ankylosing spondylitis 0 Pickwickian syndrome (marked obesity) 0

Pectus excavatum

0 KyphoScoliosis 0

Scleroderma

D. Abdominal diseaseS e.g. Ascites 11. Hyperproduction of CO2: 0 t Carbohydrate load 0 Hyperthyroid storm 0 Malignant hyperthermia 0 Severe Shivering 0

Seizures

❖ Clinical picture

0 Hyperventilation o

0 Hypoventilation with respiratory failure type H e.g.

Paresthesia & dizziness

CO2 narcosis & flapping tremors

o Tetany o

0

Features of the cause

Features of the cause

❖ Investigation o High pH 0 Low PaC02,HCO3".

❖ Treatment 0

1

0 Low pH 0 HighPaC02,HC03-.

Treatment of the cause

0 Reassurance & Rebreathing in a paper bag (not plastic

0

Treatment of the cause

0 Bronchodilators & mechanical ventilation

will cause suffocation) 0 Severe cases of alkalosis; IV HCL

40

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

I

/

Other available books by the author .Pulmonology,Hematology & Infection

.Hepatology, Gastroenterology & Endociinology .Neurology & Rheumatology .Sewilam's Internal Medicine Revision:

MCQs,Cases,Written & Oral Questions .Sewilam's Clinical Medicine

.Paraclinical Made Easy: X-Rays,Clinical pathology & ECG .Dermatology .Psychiatry

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬

‫اﺷﺗري ﻧﺳﺧﺗك إذا ﻧﻔﻌك اﻟﻛﺗﺎب وادﻋم ﺗﺣدﯾﺛﮫ ﺑﻧﯾﺔ ﻧﺷر اﻟﻌﻠم‬