Principles of cardiology total.samir rafla

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Principles of Cardiology
Samir M. Rafla, MD, FESC
Professor of Cardiology
Alexandria University

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Title: Principles of Cardiology. 2008
Author: Prof. Samir Rafla, MD, FACC, FESC
Head of the Cardiology Dept. Alexandria Univ. from 1 Aug 2004 to 30 Aug 2007
MBChB (Honor) June 1970
Printed by: Delta Center for Printing: 24 Delta street, Sporting, T. 03 5901923
Computer work: Mr. Haytham Abdel-Moneim
Distributed by: El-Sherok Library. T 03 484 8673
Address for correspondence: Prof. Samir Rafla
smrafla@hotmail.com 0101495577 03 5910170
First edition: 2008
Cover drawing by Dr. Marilyn Samir
National Number
2008/16131
Previous books by the author:
- Differential Diagnosis in Clinical Medicine. Vol. one: Heart and Chest. 1998
- Recent Advances in Diagnosis and Management of Arrhythmias. 2000
- Alexandria Book of Cardiology. Co-author with the staff of the cardiology dept.
Alexandria Univ., and co-editor with Prof. Tarek El-Badawy. 2004

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CONTENTS
Subject Page
Electrocardiography 1
Rheumatic Fever 3
Valvular Heart Disease
Mitral stenosis
The cardiac cycle
Mitral Regurgitation
Mitral valve prolapse
Aortic stenosis
Aortic Regurgitation
Tricuspid Stenosis
Tricuspid Regurgitation
6
7
13
15
16
19
21
22
Congenital Heart Disease
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
Fallot’s tetralogy
Coarctation of the aorta
Pulmonary stenosis
24
25
27
28
30
33
34
Syncope and hypotension
Sudden cardiac death
36
39
Cardiac Arrhythmias
Sick Sinus Syndrome
Premature beats (Extrasystoles)
Supraventricular Tachyarrhythmias
Atrial flutter
AV nodal reentrant tachycardia
Wolff-Parkinson-White syndrome
Atrial fibrillation
Treatment of Atrial Fibrillation
Anticoagulation for Atrial Fibrillation
Ventricular tachycardia
Long QT syndrome
Implantable Cardioverter Defibrillator (ICD)
AV heart block
Electrophysiologic study
Cardiac pacemakers
Antiarrhythmic drugs
41
42
43
44
44
46
47
48
51
53
54
55
56
56
58
59
60
Heart Failure
Right heart failure
Management
Pharmacological therapy
Ventricular Resynchronization Therapy
Acute left ventricular failure
Cardiogenic shock
62
67
69
70
74
75
77

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Refractory heart failure 80
Infective Endocarditis
Treatment
81
86
Ischemic Heart Disease
Risk factors for atherosclerosis
Stable angina pectoris
Chest pain algorithm
Unstable angina and NSTEMI
Myocardial infarction
Shock algorithm
Treatment of STEMI
Anticoagulants
87
88
89
92
95
97
103
106
110
Hypertension
Phaeochromocytoma
Investigation of the Hypertensive Patient
Treatment
Hypertensive emergencies
112
117
119
123
128
Aortic aneurysm and aortic dissection
Aortic dissection
Aortic dissection classification
129
129
132
Diseases of the peripheral arteries and veins
Deep vein thrombosis
134
135
The Lungs and Pulmonary Circulation
Pulmonary Embolism
Pulmonary Hypertension
Pulmonary Hypertension algorithm
Primary pulmonary hypertension
Cor pulmonale
Schistosomal corpulmonale
137
137
143
144
146
147
148
Diseases of The Pericardium
Acute pericarditis
Pericardial effusion
Pericardial tamponade
Constrictive pericarditis
149
149
151
152
152
Cardiomyopathy and myocarditis
Cardiomyopathy
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
Cardiac tumors
154
155
156
156
158
158
Question of the medical rounds 159

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Principles of Cardiology pages 1-61
ELECTROCARDIOGRAPHY
Prof. Samir Rafla
The electrocardiogram (ECG) is a graphic representation of the electrical activity generated by the
heart during the cardiac cycle. The electrical activity starts from the SA node, bundle of His, right and
left bundles, Purkinje fibers to stimulate the ventricles.
Waveforms: The waveforms and intervals of the ECG are: The P wave = atrial depolarization. The
QRS complex = ventricular depolarization. The Q wave is the initial downward deflection, the R wave
is the initial upward deflection, and the S wave is the second downward deflection. The interval from
the beginning of the P wave to the beginning of the Q wave is the PR interval.
The T wave = ventricular repolarization. The interval from the end of ventricular depolarization to the
beginning of the T wave is termed the ST segment. The interval from the onset of ventricular
depolarization to end of T is the QT interval.
STANDARD APPROACH TO THE ECG: Normally, standardization is 1.0 mV per 10 mm, and
paper speed is 25 mm/s (each horizontal small box = 0.04 sec)
Heart Rate: divide 1500 by number of small boxes between each QRS.
Rhythm: Sinus rhythm is present if every P wave is followed by a QRS, PR interval > 0.12 s, and the P
wave is upright in leads I, II, and III.
Intervals: PR (0.12 - 0.20 s). QRS (0.06 - 0.10 s).
QT 0.43 s;
ST-T WAVES: ST elevation : Acute MI, coronary spasm, pericarditis (concave upward), LV
aneurysm.
ST depression: Digitalis effect, strain (due to ventricular hypertrophy), ischemia, or nontransmural MI.
Tall peaked T: Hyperkalemia; acute MI ("hyperacute T").
Inverted T: Non-Q-wave MI, ventricular "strain" pattern, drug effect (e.g., digitalis), hypokalemia,
hypocalcemia, increased intracranial pressure (e.g., subarachnoid bleeding).

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FIG: The magnified ECG wave is presented with the principal time intervals indicated.
Fig: The pathways of Conduction.

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RHEUMATIC FEVER
Introduction. Classified as a connective tissue or collagen vascular disease, rheumatic fever (RF) is
the leading cause of acquired heart disease in children and young adults.
a. In many developing countries the incidence of acute RF approaches or exceeds 100 per 100.000,
whereas in the Unites States it is estimated to be less than 2 per 100.000.
b. Rheumatic fever is more common among population at high risk for streptococcal pharyngitis, those
in close contact with school age children, and persons of low socioeconomic status. It occurs
commonly between the ages of 5 and 18 years and is rare before 5. Rheumatic fever affects both sexes
equally, except for Sydenham’s chorea, which is more prevalent in females after puberty.
The clinical manifestations of RF develop after a silent period of approximately 3 weeks following a
tonsillopharyngitis caused by a group A streptococcal infection (GAS).
Diagnostic criteria
1. The Jones criteria, are designed to aid in the diagnosis of the first episode of RF. Rheumatic
fever can be diagnosed when a previous upper airway infection with GA-Streptococci is detected in
conjunction with either two major manifestations, or one major and two minor manifestations. Major
manifestation includes arthritis, carditis, chorea, erythema marginatum, and subcutaneous nodules.
Minor manifestations include: fever, arthralgias, history of tonsillitis 1-3 weeks before the arthralgia,
history of rheumatic heart disease;
high C-reactive protein, high erythrocyte sedimentation rate, raised antistreptolysin O titer above 200
Todd’s units or prolonged PR interval on electrocardiogram (ECG).
Major manifestations:
1. Carditis: affecting 41% to 83% of patients. It can be defined as pancarditis affecting the
endocardium, myocardium, and pericardium: The main clinical manifestations include increased heart
rate, murmurs, cardiomegaly, rhythm disturbances, pericardial friction rub, and heart failure.
Congestive heart failure is rare in the acute phase; if present, it usually results from myocarditis. The
most characteristic component of rheumatic carditis is a valvulitis (endocarditis) involving the mitral
and aortic valves.

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Pericarditis may cause chest pain, friction rubs, and distant heart sounds.
2. Arthritis. This is the most common manifestation of RF. It is present in around 80% of the patients
and has been described as painful, asymmetric, migratory, and transient; it involves large joints, such as
knees, ankles, elbows, wrists, and shoulders. It improves markedly with the use of salicylates within 48
hours of treatment. Monoarthritis, oligoarthritis, and involvement of small joints of the extremities are
less common. The arthritis of RF is benign and self- limiting (Lasting 2 to 3 weeks) and does not result
in permanent sequelae.
3. Sydenham’s chorea. This extrapyramidal disorder is characterized by purposeless and involuntary
movements of face and limbs, muscular hypotonia, and emotional lability.
4. Subcutaneous nodules.
5. Erythema marginatum.
Minor manifestations:
1. Fever is encountered during the acute phase of the disease.
2. Arthralgia is defined as pain in one or more large joints without objective findings of inflammation
on physical examination.
3. Other clinical manifestations of RF include abdominal pain, epistaxis, acute glomerulonephritis.
These are not included as diagnostic criteria for the diagnosis of RF.
Laboratory examination and diagnostic testing.
1. Neither throat culture nor rapid antigen test, if positive; differentiate
between recent infection associated with RF and chronic carriage of
pharyngeal GAS.
2. Antistreptolysin O is the most commonly available test. Elevated or rising ASO titers provide solid
evidence for recent GAS infection. A greater than two-fold rise in ASO titers compared with
convalescent titers is diagnostic.
3. Increased sedimentation rate.
4. Increased C reactive protein CRP/
5. The most common finding in the electrocardiogram is the presence of P-R prolongation and sinus
tachycardia.
Therapy:

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Patient with the diagnosis of rheumatic activity should initially receive a full course of antibiotic to
ensure proper eradication of the organism.
A. Arthritis: Anti-inflammatory medications are generally recommended for 3 weeks for symptomatic
relief.
1. Pain resolves within 24 hours of starting therapy with salicylates.
2. If pain persists after salicylate treatment, the diagnosis of RF is questionable.
3. The recommended dose of salicylate is 100 mg/kg per day, given in 4 divided doses. Toxic effects
such as anorexia, nausea, vomiting, and tinnitus should be avoided.
B. Carditis
1. Strenuous physical activity should be avoided.
2. Congestive heart failure should be treated with appropriate therapy.
3. In patients with significant cardiac involvement, corticosteroids are preferred over salicylates. The
recommended dose is 1 to 2 mg/kg per day, (maximum of 60 mg/day as Prednisolone). Commonly,
therapy is needed for more than one month in patients with cardiac involvement. Therapy should be
continued until there is sufficient clinical and laboratory evidence of disease inactivity.
4. The gradual reduction in steroid doses is important to avoid relapses. Use of salicylates (75 mg/kg
per day) while tapering corticosteroids may reduce the likelihood a relapse.
Summary: Jones Criteria of Rheumatic Fever
Major Criteria Minor Criteria
Migratory polyarthritis Fever
Carditis Arthralgia
Chorea High sedimentation rate
Subcutaneous nodules Positive C reactive protein
Erythema Marginatum Prolonged PR interval
Prevention:
The most important step in the treatment of RF is the eradication of GAS infection.
Penicillin is the agent of choice. A. best results are achieved with a single intramuscular dose of
penicillin G benzathine. b. The oral antibiotic of choice is penicillin V (phenoxymethyl penicillin) (see
Table for dosage information). Patients allergic to penicillin: oral erythromycin can be used. The
recommended dosage is erythromycin for 10 days. The maximal dose of erythromycin is 1 g/day.

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Table: Duration of therapy for secondary prevention of rheumatic fever
Disease state Duration of therapy
RF + carditis + residual valvular
disease
At least 10 years post episode and at least
until age 40. Lifelong prophylaxis may be
required
RF + carditis without valvular
disease
10 years or beyond adulthood, whichever
is longer.
RF without carditis 5 years or until age of 21, whichever is
longer.
RF, rheumatic fever.
VALVULAR HEART DISEASE
MITRAL STENOSIS
ETIOLOGY AND PATHOLOGY: Two-thirds of all patients with mitral stenosis (MS) are females.
MS is generally rheumatic in origin. Pure or predominant MS occurs in approximately 40% of all
patients with rheumatic heart disease. The valve leaflets are diffusely thickened by fibrous tissue and/or
calcific deposits. The mitral commissures fuse, the chordae tendineae fuse and shorten. The valvular
cusps become rigid, and these changes in turn, lead to narrowing at the apex of the funnel-shaped
valve.
Other rare causes of mitral stenosis: Atrial myxoma, ball valve thrombus, congenital and calcific-
atherosclerortic disease.
PATHOPHYSIOLOGY: In normal adults the mitral valve orifice is 4 to 6 cm2
. When the mitral valve
opening is reduced to 1 cm2
, a left atrial pressure of approximately 25 mmHg is required to maintain a
normal cardiac output. The elevated left atrial pressure, in turn, raises pulmonary venous and capillary
pressures, reducing pulmonary compliance and causing exertional dyspnea.
Pulmonary hypertension results from (1) the passive backward transmission of the elevated left atrial
pressure, (2) pulmonary arteriolar constriction, (reactive pulmonary hypertension), and (3) organic
obliterative changes in the pulmonary vascular bed. In time, the resultant severe pulmonary
hypertension results in tricuspid and pulmonary incompetence as well as right-sided heart failure.

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SYMPTOMS AND COMPLICATIONS: - Dyspnea, hemoptysis. - Orthopnea and paroxysmal
nocturnal dyspnea. Pulmonary edema develops when there is a sudden surge in flow across a markedly
narrowed mitral orifice.
The cardiac cycle: Simultaneous electrocardiogram and pressure obtained from the left atrium, left
ventricle, and aorta, and the jugular pulse during one cardiac cycle.
When moderately severe MS has existed for several years, atrial arrhythmias as flutter and fibrillation
occur.
Hemoptysis results from rupture of pulmonary-bronchial venous connections (apoplexy) secondary to
pulmonary venous hypertension. Frank hemoptysis must be distinguished from the bloody sputum that
occurs with pulmonary edema, pulmonary infarction, and bronchitis, three conditions that occur with
increased frequency in the presence of MS.
Recurrent pulmonary emboli, sometimes with infarction are an important cause of morbidity and
mortality late in the course of MS, occurring most frequently in patients with right ventricular failure.

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Pulmonary infections, i.e., bronchitis, broncho-pneumonia, and lobar pneumonia, commonly
complicate untreated MS. Infective endocarditis is rare in pure MS but is not uncommon in patients
with combined stenosis and regurgitation.
Summary: Causes of hemoptysis in mitral stenosis:
- Bronchitis
- Congestion
- Pulmonary edema
- Pulmonary embolism, infarction
- Pulmonary apoplexy
Thrombi and emboli: Thrombi may form in the left atrium, particularly in the enlarged atrial
appendage of patients with MS. If they embolize, they do so most commonly to the brain, kidneys,
spleen, and extremities. Embolization occurs much more frequently in patients with atrial fibrillation.
Rarely, a large pedunculated thrombus or a free-floating clot may suddenly obstruct the stenotic mitral
orifice. Such “ball valve” thrombi produce syncope, angina, and changing auscultatory signs with
alterations in position, findings that resemble those produced by a left atrial myxoma.
PHYSICAL FINDINGS: Inspection: In advanced cases there is a malar flush. When fibrillation is
present, the jugular pulse reveals only a single expansion during systole (c-v wave) (systolic venous
pulse).
Palpation: Left parasternal lift along the left sternal border signifies an enlarged right ventricle. In
patients with pulmonary hypertension, the impact of pulmonary valve closure can usually be felt in the
second and third left intercostal spaces just left of the sternum (Diastolic shock). A diastolic thrill is
frequently present at the cardiac apex, particularly if the patient is turned into the left lateral position.
Auscultation: The first heart sound (S1) is generally accentuated and snapping. In patients with
pulmonary hypertension, the pulmonary component of the second heart sound (P2) is often accentuated,
and the two components of the second heart sound are closely split. The opening snap (OS) of the
mitral valve is most readily audible in expiration at, or just medial to, the cardiac apex but also may be
easily heard along the left sternal edge. This sound generally follows the sound of aortic valve closure
(A2) by 0.05 to 0.12; that is, it follows P2; the time interval between A2 closure and OS varies inversely
with the severity of the MS. It tends to be short (0.05 to 0.07 s) in patients with severe obstruction, and

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long, (0.10 to 0.12 s) in patients with mild MS. The intensities of the OS and S1 correlate with mobility
of the anterior mitral leaflet.
The OS usually precedes a low-pitched, rumbling, diastolic murmur, heard best at the apex with the
patient in the left lateral recumbent position. In general, the duration of the murmur correlates with the
severity of the stenosis. In patients with sinus rhythm, murmur often reappears or becomes accentuated
during atrial systole, as atrial contraction elevates the rate of blood flow across the narrowed orifice
(presystolic accentuation).
Associated lesion: With severe pulmonary hypertension, a pansystolic murmur produced by
functional tricuspid regurgitation may be audible along the left sternal border. Characteristically, this
murmur is accentuated by inspiration, and should not be confused with the apical pansystolic murmur
of mitral regurgitation.
In the presence of severe pulmonary hypertension and right ventricular failure, a third heart sound
may originate from the right ventricle. The enlarged right ventricle may rotate the heart in a clockwise
direction and form the cardiac apex, giving the examiner the erroneous impression of left ventricular
enlargement. Under these circumstances, the rumbling diastolic murmur and the other auscultatory
features of MS become less prominent or may even disappear and be replaced by the systolic murmur
of functional tricuspid regurgitation which is mistaken for mitral regurgitation. When cardiac output is
markedly reduced in a patient with MS, the typical auscultatory findings, including the diastolic
rumbling murmur, may not be detectable (silent MS).
ECG findings: The P wave is wide and may be notched which suggests left atrial enlargement. It
becomes tall and peaked in lead II and upright in lead V1 when severe pulmonary hypertension.

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Echocardiogram: Two-dimensional echo-Doppler echocardiography for estimation of the
transvalvular gradient and of mitral orifice size, the presence and severity of accompanying mitral
regurgitation, the extent of restriction of valve leaflets, their thickness, and the subvalvular changes.
Transthoracic and transesophageal echo are needed to verify presence of atrial thrombi.
X-Ray chest: Straightening of the left border of the cardiac silhouette, prominence of the main
pulmonary arteries, dilatation of the upper lobe pulmonary veins, and backward displacement of the
esophagus by an enlarged left atrium.
Summary of signs of mitral stenosis:
- Mid-diastolic rumbling murmur with presystolic accentuation;
- Snappy first sound;
- Opening snap;
- Diastolic thrill.
DIFFERENTIAL DIAGNOSIS: The apical middiastolic murmur associated with aortic regurgitation
(Austin Flint murmur) may be mistaken for MS. However, in a patient with aortic regurgitation, the
absence of an opening snap or presystolic accentuation if sinus rhythm is present points to the absence
of MS.
Tricuspid stenosis, a valvular lesion that occurs very rarely in the absence of MS, may mask many of
the clinical features of MS.
MANAGEMENT: Penicillin prophylaxis of beta-hemolytic streptococcal infections and prophylaxis
for infective endocarditis are important. In symptomatic patients, some improvement usually occurs
with restriction of sodium intake and maintenance doses of oral diuretics. Digitalis glycosides usually
do not benefit patients with pure stenosis and sinus rhythm, but they are necessary for slowing the
ventricular rate of patients with atrial fibrillation and for reducing the manifestations of right-sided
heart failure in the advanced stages of the disease.
Small doses of beta-blockers (e.g., atenolol 25 mg/d) may be added when cardiac glycosides fail to
control ventricular rate in patients with atrial fibrillation. Particular attention should be directed toward
detecting and treating any accompanying anemia and infections. Hemoptysis is treated by measures
designed to diminish pulmonary venous pressure, including bed rest, the sitting position, salt

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restriction, and diuresis. Anticoagulants should be administered continuously in those with atrial
fibrillation.
If atrial fibrillation is of relatively recent origin in a patient who’s MS is not severe enough to warrant
surgical treatment, reversion to sinus rhythm pharmacologically or by means of electrical countershock
is indicated. Usually this should be undertaken following 3 weeks of anticoagulant treatment.
Conversion to sinus rhythm is rarely helpful in patients with severe MS, particularly those in whom the
left atrium is especially enlarged or in whom atrial fibrillation is chronic.
Mitral valvotomy by balloon or surgical mitral valvotomy, is indicated in the symptomatic patient with
pure MS whose effective orifice is less than approximately 1.3 cm2
(or 0.8 cm2
/ m2
of body surface
area). Mitral valve replacement by prosthetic valve is resorted to only if the valve is heavily calcified
and associated with incompetence.
Percutaneous balloon valvuloplasty is an alternative to surgical mitral valvuloplasty in patients with
pure or predominant rheumatic stenosis (it is now the first choice). Young patients without extensive
valvular calcification or thickening or subvalvular deformity are the best candidates for this procedure.
Contraindications of balloon mitral valvotomy:
1. presence of left atrial thrombi,
2. presence of combined mitral incompetence and stenosis, and
3. heavily calcified mitral cusps.
MITRAL REGURGITATION
ETIOLOGY:
1- Chronic rheumatic heart disease is the cause of severe mitral regurgitation (MR).
2- MR also may occur as a congenital anomaly.
3- MR may occur in patients with infarction involving the base of a papillary muscle.
4- MR may occur with marked left ventricular dilatation.
5- Massive calcification of the mitral annulus of unknown cause, presumably degenerative, which
occurs most commonly in elderly women.
6- Systemic lupus erythematosus, rheumatoid arthritis, are less common cause.

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7- Mitral prolapse.
Acute MR occur 1- secondary to infective endocarditis involving the cusps or chordae tendineae, 2- in
acute myocardial infarction with rupture of a papillary muscle or one of its heads, 3- as a consequence
of trauma, 4- or following apparently spontaneous chordal rupture.
MITRAL REGURGITATION: SYMPTOMS: Fatigue, exertional dyspnea, and orthopnea are the
most prominent complaints in patients with chronic, severe MR. Hemoptysis and systemic
embolism also occur less frequently in MR than in MS. Right-sided heart failure, with painful
hepatic congestion, ankle edema, distended neck veins, ascites, and tricuspid regurgitation,
may be observed in patients with MR who have associated pulmonary vascular disease and
marked pulmonary hypertension. In patients with acute, severe MR, left ventricular failure
with acute pulmonary edema and /or cardiovascular collapse is common.
PHYSICAL FINDINGS: Palpation: A systolic thrill is often palpable at the cardiac apex, the left
ventricle is hyperdynamic, and the apex beat is often displaced laterally. Auscultation: The first heart
sound is generally absent, soft (muffled), or buried in the systolic murmur. A low-pitched third heart
sound (S3) occurring 0.12 to 0.17 sec after aortic valve closure, i.e. at the completion of the rapid-filling
phase of the left ventricle, is an important auscultatory feature of severe MR.
A fourth heart sound is often audible in patients with acute, severe MR of recent onset who are in sinus
rhythm. A systolic murmur of at least grade III/VI intensity is the most characteristic auscultatory
finding in severe MR. It is usually holosystolic (pansystolic). In MR due to papillary muscle
dysfunction or mitral valve prolapse, the systolic murmur commences in midsystole. In patients with
ruptured chordae tendineae the systolic murmur may have a cooing or “sea gull” quality; in patients
with a flail leaflet the murmur may have a musical quality.
Summary: Signs of mitral incompetence:
- Harsh pansystolic murmur over apex propagated to axilla.
- Muffled first heart sound.
- Systolic thrill over apex.

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Electrocardiogram: In patients with sinus rhythm there is evidence of left atrial enlargement (P
mitrale), but right atrial enlargement also may be present when pulmonary hypertension is severe.
Chronic, severe MR with left atrial enlargement is generally associated with atrial fibrillation.
Echocardiogram: Doppler echocardiography and color Doppler flow echocardiography imaging are the
most accurate noninvasive techniques for the detection and estimation of MR. The left atrium is usually
enlarged. Findings which help to determine the etiology of MR can often be identified; these include
vegetations associated with infective endocarditis, incomplete coaptation of the anterior and posterior
mitral leaflets, and annular calcification, as well as left ventricular dilation, aneurysm, or dyskinesia.
The echocardiogram in patients with mitral valve prolapse is described below.
Roentgenogram: The left atrium and left ventricle are the dominant chambers; in chronic cases, the
former may be massively enlarged and forms the right border of the cardiac silhouette. Pulmonary
venous congestion, interstitial edema, and Kerly B lines are sometimes noted.
TREATMENT: Medical: The non surgical management of MR is directed toward restricting those
physical activities that regularly produce dyspnea and excessive fatigue, reducing sodium intake, and
enhancing sodium excretion with the appropriate use of diuretics. Vasodilators and digitalis glycosides
increase the forward output of the failing left ventricle. Angiotensin-converting enzyme inhibitors are
given in chronic MR. The same considerations as in patients with MS apply to the reversion of atrial
fibrillation to sinus rhythm. Surgical treatment should be offered to patients with severe MR whose
limitations do not allow them to perform normal household activities despite optimal medical
management. Surgery is indicated when the end systolic diameter of the left ventricle by echo exceeds
50 mm.
MITRAL VALVE PROLAPSE
Mitral valve prolapse (MVP), also termed the systolic click-murmur syndrome, is a common, but
highly variable, clinical syndrome. It is a frequent finding in patients who have the typical features of
the Marfan syndrome. The posterior leaflet is usually more affected than the anterior, and the mitral
valve annulus is often greatly dilated.
MVP may be associated with thoracic skeletal deformities.
MVP is common in females between the ages of 6 and 30 years. Most patients are asymptomatic and
remain so for their entire lives. Arrhythmia, most commonly ventricular premature contractions and

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paroxysmal supraventricular and ventricular tachycardia, have been reported and may cause
palpitations, light-headedness, and syncope. Many patients have chest pain which is difficult to
evaluate.
PHYSICAL EXAMINATION: Auscultation: the most important finding is the mid-or late
(nonejection) systolic click, which occurs 0.14 s or more after the first heart sound. Systolic clicks may
be followed by a high-pitched late systolic murmur, heard best at the apex. A useful echocardiographic
definition of MVP is systolic displacement (in the parasternal view) of the mitral valve leaflets into the
left atrium > 3 mm. Thickening of the mitral valve leaflets is present. Doppler studies are helpful in
revealing and evaluating accompanying MR.
Treatment: The management of patients with MVP consists of reassurance of the asymptomatic
patient without severe MR or arrhythmias; prevention of infective endocarditis with antibiotic
prophylaxis in patients with a systolic murmur and the relief of the atypical chest pain by beta blockers.
AORTIC STENOSIS
Aortic stenosis (AS) occurs in one-fourth of all patients with chronic valvular heart disease;
approximately 80 percent of adult patients with symptomatic valvular AS are male.
Etiology: 1. AS may be congenital in origin, 2. secondary to rheumatic inflammation of the valve, 3.
degenerative calcification of the aortic cusps of unknown cause.
PATHOPHYSIOLOGY: A peak systolic pressure gradient exceeding 50 mmHg or an effective aortic
orifice less than approximately 0.5 cm2
/m2
of body surface area i.e., less than approximately one-third
of the normal orifice, is generally considered to represent critical obstruction to left ventricular outflow.
SYMPTOMS: AS is rarely of hemodynamic or clinical importance until the valve orifice has narrowed
to approximately one-third of normal, i.e., to 1 cm2
in adults.
Exertional dyspnea, angina pectoris, and syncope are the three cardinal symptoms. Angina pectoris
reflects an imbalance between the augmented myocardial oxygen requirement by the hypertrophied
myocardium and the un-accompanying increase in coronary blood flow. Orthopnea, paroxysmal
nocturnal dyspnea, and pulmonary edema, i.e., symptoms of left ventricular failure, also occur only in
the advanced stages of the disease.
PHYSICAL FINDINGS: A palpable double systolic arterial pulse the so-called bisferiens pulse,
excludes pure or predominant AS and signifies dominant or pure aortic regurgitation or obstructive
hypertrophic cardiomyopathy.

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Palpation: The apex beat is usually sustained and displaced laterally, reflecting the presence of left
ventricular hypertrophy. A systolic thrill is generally present at the base of the heart in the suprasternal
notch, and along the carotid arteries.
Auscultation: Harsh ejection systolic murmur over aortic area propagated to carotids. The sound of
aortic valve closure, the second sound is very weak or even absent with tight aortic stenosis.
Frequently, a fourth heart sound is audible at the apex in many patients with severe AS and reflects
the presence of left ventricular hypertrophy and an elevated left ventricular enddiastolic pressure; a
third heart sound generally occurs when the left ventricle dilates and fails.
The murmur of AS is characteristically an ejection systolic murmur loudest at the base of the
heart, most commonly in the second right intercostal space. It is transmitted along the carotid arteries.
Occasionally, it is transmitted downward and to the apex and may be confused with the systolic
murmur of MR.
Summary: Signs of aortic stenosis:
1. Harsh ejection systolic murmur over aortic area propagated to carotids.
2. Weak or absent second heart sound (aortic component)
3. Systolic thrill over aortic area, suprasternal notch and carotids.
4. Strong sustained apex,
Electrocardiogram: This reveals left ventricular hypertrophy in the majority of patients with severs
AS.
Echocardiogram: The key findings are left ventricular hypertrophy. The transaortic valvular gradient
can be estimated by Doppler echocardiography.
Congestive heart failure was considered to be the cause of death in one-half to two-thirds of patients.
Among adults dying with valvular AS sudden death, which presumably results from an arrhythmia
(ventricular tachycardia or fibrillation) occurred in 10 to 20 percent and at an average age of 60 years.
TREATMENT: All patients with moderate or severe AS require careful periodic follow-up. In
patients with severe AS, strenuous physical activity should be avoided even in the asymptomatic stage.
Digitalis glycosides, sodium restriction, and the cautious administration of diuretics are indicated in the
treatment of congestive heart failure, but care must be taken to avoid volume depletion.
In the majority of adults with calcific AS and critical obstruction, replacement of the valve is necessary.
Percutaneous balloon aortic valvuloplasty is an alternative to surgery in children and young adults with

21
congenital aortic stenosis. It is not commonly employed in elderly with severe calcific aortic stenosis
because of a high restenosis rate.
Electrocardiogram (ECG), left ventricular, and aortic pressure curves in a patient with aortic stenosis.
There is a pressure gradient across the aortic valve during systole

22
Fig. Abnormal sounds and murmurs associated with valvular dysfunction displayed simultaneously
with left atrial (LA), left ventricular (LV), and aortic pressure tracings. AVO, aortic valve opening; E,
ejection click; MVO, mitral valve opening; OS, opening snap of the mitral valve.
.
AORTIC REGURGITATION
ETIOLOGY: Approximately three-fourths of patients with pure or predominant aortic regurgitation
(AR) are males; females predominate among patients with AR who have associated mitral valve
disease.
Causes:
1- In approximately two-thirds of patients with AR the disease is rheumatic in origin, resulting in
thickening, deformation and shortening of the individual aortic valve cusps, changes which prevent
their proper opening during systole and closure during diastole.
2- Acute AR also may result from infective endocarditis, which may attack a valve previously affected
by rheumatic disease, a congenitally deformed valve, or rarely a normal aortic valve, and perforate or
erode one or more of the leaflets.
3- Patients with discrete membranous subaortic stenosis often develop thickening of the aortic valve
leaflets, which in turn leads to mild or moderate degrees of AR.
4- AR also may occur in patients with congenital bicuspid aortic valves.
5- Aortic dilatation, i.e., aortic root disease, widening of the aortic annulus and separation of the aortic
leaflets are responsible for the AR.
6- Syphilis and ankylosing rheumatoid spondylitis may lead to aortic dilatation, aneurysm formation,
and severe regurgitation.
7- Cystic medial necrosis of the ascending aorta, associated with other manifestations of the Marfan
syndrome, idiopathic dilatation of the aorta, and severe hypertension all may widen the aortic annulus
and lead to progressive AR.
8- Occasionally, AR is caused by retrograde dissection of the aorta involving the aortic annulus.

23
History: Patients with severe AR may remain asymptomatic for 10 to 15 years.
Sinus tachycardia during exertion may produce particularly uncomfortable palpitations. Exertional
dyspnea is the first symptom of diminished cardiac reserve. This is followed by orthopnea, paroxysmal
nocturnal dyspnea, and excessive diaphoresis. Chest pain occurs frequently, even in younger patients,
due to diminished coronary filling during diastole.
Nocturnal angina may be a particularly troublesome symptom. The anginal episodes can be prolonged
and often do not respond satisfactorily to sublingual nitroglycerin. Late in the course of the disease,
evidence of systemic fluid accumulation, including congestive hepatomegaly, ankle edema, and ascites,
may develop.
PHYSICAL FINDINGS: Peripheral signs: Arterial pulse: A rapidly rising “water-hammer” pulse,
which collapses suddenly as arterial pressure falls rapidly during late systole and diastole, and capillary
pulsations, an alternate flushing and paling of the root of the nail while pressure is applied to the tip of
nail, are characteristic of free AR. A booming, “pistol-shot” sound can be heard over the femoral or
brachial arteries, and a to - fro murmur is audible if the femoral artery is lightly compressed with a
stethoscope.
The arterial pulse pressure is widened, with an elevation of the systolic pressure and a depression of the
diastolic pressure. The severity of AR does not always correlate directly with the arterial pulse
pressure, and severe regurgitation may exist in patients with arterial pressures in the range of 140/60.
Palpation: The apex beat is strong and displaced laterally and inferiorly. The systolic expansion and
diastolic retraction of the apex are prominent and contrast sharply with the sustained systolic thrust
characteristic of severe AS. In many patients with pure AR or with combined AS and AR, palpation or
recording of the carotid arterial pulse reveals it to be bisferiens, i.e., with two systolic waves separated
by trough.
Auscultation: A third heart sound is common, and occasionally, a fourth heart sound also may be heard.
The murmur of AR is typically a high-pitched, blowing, decrescendo early diastolic murmur which is
usually heard best in the third left intercostal space. Unless it is trivial in magnitude, the AR is usually
accompanied by peripheral signs such as a widened pulse pressure or a collapsing pulse. On the other
hand, with the Graham steel murmur of pulmonary regurgitation, there is usually clinical evidence of
severe pulmonary hypertension, including a loud and palpable pulmonary component to the second
heart sound.

24
A midsystolic ejection murmur is frequently audible in AR. It is generally heard best at the base of
the heart and is transmitted to the carotid vessels. This murmur may be quite loud without signifying
organic obstruction; it is often higher pitched, shorter, than the ejection systolic murmur heard in
patients with predominant AS.
A third murmur which is frequently heard in patients with AR is the Austin Flint murmur, a soft,
low-pitched, rumbling middiastolic or presystolic bruit. It is probably produced by the displacement of
the anterior leaflet of the mitral valve by the aortic regurgitant stream. Both the Austin Flint murmur
and the rumbling diastolic murmur of MS are loudest at the apex, but the murmur of MS is usually
accompanied by a loud first heart sound and immediately follows the opening snap of the mitral valve,
while the Austin Flint murmur is often shorter in duration than the murmur of MS, and in patients with
sinus rhythm the latter exhibits presystolic accentuation.
Summary: Signs of aortic incompetence over the heart:
- Soft blowing early diastolic murmur over aortic area propagated to apex.
- Austin-Flint murmur (diastolic murmur over mitral area).
Echocardiogram: Essential for detection of severity and cause of AR.
TREATMENT: Although operation constitutes the principal treatment of aortic regurgitation, and
should be carried out before the development of heart failure, the latter usually respond initially to
treatment with digitalis, salt restriction, diuretics, and vasodilators, especially angiotensin-converting
enzyme inhibitors.
In patients with severe AR, careful clinical follow-up and noninvasive testing with echocardiography at
approximately 6-month intervals are necessary. Operation is to be undertaken at the optimal time, i.e.,
after the onset of left ventricular dysfunction but prior to the development of severe symptoms. Valve
replacement is indicated if the LV dilates to 50 mm in systole and 65 to 70 mm in diastole.
ACUTE AORTIC REGURGITATION: Infective endocarditis, aortic dissection, and trauma are the
most common causes of severe, acute AR.
TRICUSPID STENOSIS

25
It is generally rheumatic in origin and is more common in women than in men. It does not usually
occur as an isolated lesion or in patients with pure MR but is usually observed in association with MS.
Hemodynamically significant TS occurs in 5 to 10 percent of patients with severe MS; rheumatic TS is
commonly associated with some degree of regurgitation.
SYMPTOMS: Since the development of MS generally precedes that of TS, many patients initially have
symptoms of pulmonary congestion. Amelioration of the latter should raise the possibility that TS may
be developing. Fatigue secondary to a low cardiac output and discomfort due to refractory edema,
ascites, and marked hepatomegaly are common in patients with TS and / or regurgitation.
Severe TS is associated with marked hepatic congestion, often resulting in cirrhosis, jaundice,
serious malnutrition, anasarca, and ascites. The jugular veins are distended, and in patients with sinus
rhythm there may be giant “a” waves.
On auscultation, the pulmonic closure sound is not accentuated, and occasionally, an OS of the
tricuspid valve may be heard approximately 0.06 s after pulmonic valve closure. The diastolic murmur
of TS has many of the quality of the diastolic murmur of MS, and since TS almost always occurs in the
presence of MS, the less common valvular lesion may be missed. The murmur is augmented during
inspiration, and it is reduced during expiration.
Surgical treatment of the tricuspid valve is not ordinarily indicated at the time of mitral valve
surgery in patients with mild TS. On the other hand, definitive surgical relief of the TS should be
carried out, preferable a the time of mitral valvotomy, in patients with moderate or severe TS who have
mean diastolic pressure gradients exceeding 4 to 5 mmHg and tricuspid orifices less than 1.5 to 2.0
cm2
. TS is almost always accompanied by significant tricuspid regurgitation.
TRICUSPID REGURITATION
Most commonly, tricuspid regurgitation (TR) is functional and secondary to marked dilatation of
the right ventricle and the tricuspid annulus. Functional TR may complicate right ventricular
enlargement of any cause, including inferior wall infarcts that involve the right ventricle, and is
commonly seen in the late stages of heart failure due to rheumatic or congenital heart disease with
severe pulmonary hypertension, as well as in ischemic heart disease, cardiomyopathy, and cor
pulmonale. It is in part reversible if pulmonary hypertension is relieved. Rheumatic fever may produce
organic TR, often associated with TS. Endomyocardial fibrosis, infective endocarditis may produce
TR.

26
The clinical features of TR result primarily from systemic venous congestion and reduction of
cardiac output. The neck veins are distended with prominent V waves, and marked hepatomegaly,
ascites, pleural effusions, edema, systolic pulsations of the liver and positive hepato-jugular reflux are
common. A prominent right ventricular pulsation along the let parasternal region and a blowing
holosystolic murmur along the lower left sternal margin which may be intensified during inspiration
and reduced during expiration or the Valsalva maneuver are characteristic findings; AF is usually
present.
Summary: Signs of tricuspid regurgitation
- Pansystolic murmur over tricuspid area increases with inspiration.
- Systolic neck vein pulsations
Echocardiography and Doppler: for detection of severity of TR, estimation of pulmonary pressure and
search for vegetations of infective endocarditis.
Treatment of the underlying cause of heart failure usually reduces the severity of functional TR. In
patients with mitral valve disease and TR due to pulmonary hypertension and massive RV enlargement,
effective surgical correction of the mitral valve abnormality results in lowering of the pulmonary
vascular pressure and gradual reduction or disappearance of the TR. Tricuspid valvuloplasy by De
Vega procedure and Carpentier ring can be done.
Pulmonary Stenosis: See congenital pulmonary stenosis
Pulmonary Regurgitation
Dilatation of the pulmonary artery in cases of pulmonary hypertension may produce pulmonary
regurgitation. This is called Graham Steel murmur. It is differentiated from the early diastolic
murmur of aortic regurgitation by the associated signs of pulmonary hypertension, and by Doppler
study.

27
CONGENITAL HEART DISEASE
Congenital heart malformations remain one of the most frequent birth defects, with a live-born
prevalence of about 8 per 1000 live-born infants in western countries.
Etiology of congenital heart disease:
It is generally an abnormal form of cardiac development in the first 6-8 weeks of intrauterine life. It is
either due to exposure of the fetus in this period to injurious teratogenic factor or to abnormal
chromosomal structure.
Some causes could be identified as:
1- Drugs e.g. thalidomide, excess alcohol intake, anticonvulsant drugs.
2- Exposure to radiation e.g. X-rays and gamma rays.
3- Hereditary diseases: Diseases caused by chromosomal abnormalities eg Turner syndrome, Down
syndrome or mongolism.
4- Maternal infections e.g. German measles in the first trimester of pregnancy.
Congenital heart diseases in the adults could be classified into:
I- Left or right ventricular outflow obstruction: Aortic stenosis, pulmonary stenosis, coarctation of
aorta.
II- Left to right shunts: ASD, VSD and PDA.
III- Cyanotic heart disease: Fallot’s tetralogy and other cyanotic congenital diseases.
LEET TO RIGHT SHUNT
When there is a congenital communication between both sides of the heart, e.g. atrial or ventricular
septal defects or patent ductus arteriosus the blood always flows from the left side (left atrium, left
ventricle or aorta) to right side (right atrium, right ventricle or pulmonary artery). This is because the
pressure in all left-sided chambers is higher than in right-sided chambers.
EFFECTS:
1- Left to right shunt results in pulmonary plethora (increased vascularity in the lung). If the shunt is
very big heart failure may occur but this is rare.

28
2- In mild to moderate cases the pulmonary vessels dilate to accommodate the excessive blood flow.
Mild cases are well tolerated but if the shunt is excessive the pulmonary vessels react by
vasoconstriction. Pulmonary arteriolar vasoconstriction causes pulmonary hypertension which results
in right ventricular hypertrophy.
3- Pulmonary hypertension causes rise of pressure in the chambers of the right side of heart. Ultimately
the pressure in the right side exceeds that of the left side and the blood starts to flow across the defect
in the reverse direction, i.e. right to left shunt (reversed shunt). The patient becomes cyanosed. Emboli
originating in the venous side may be shunted across the defect to the arterial side and settle in organs
such as the brain or limbs. This is paradoxical embolism.
Closure of the defect at this stage is useless and dangerous. This situation of a congenital defect +
reversed shunt is called Eisenmenger’s syndrome. Eisenmenger’s syndrome is not an independent
congenital heart disease. It is the end result of big left to right shunt. At this stage the clinical picture is
that of central cyanosis with severe pulmonary hypertension.
ATRIAL SEPTAL DEFECT
In the presence of a defect in the atrial septum the right atrium receives blood both from the normal
venous return and the left atrium, the right atrium dilates. This results in: Dilatation and hypertrophy of
the right ventricle (volume overload), dilatation of the pulmonary artery, and pulmonary plethora. If the
defect is big and uncorrected pulmonary arteriolar vasoconstriction progressively occurs and results in
pulmonary hypertension usually at age 20-30 years. When the pressure in the right atrium exceeds that
in the atrium the shunt becomes reversed (Eisenmenger’s syndrome) and the patient becomes cyanosed.
Clinical features:
1- Atrial septal defect is more common in females. When the left to right shunt is very big pulmonary
plethora may predispose to repeated chest infections in infancy. Otherwise there are no symptoms for
many years. Ultimately heart failure occurs.
2- Atrial fibrillation occurs in late cases.
3- Right ventricular dilatation and hypertrophy cause a hyperdynamic impulse in the third and fourth
spaces to the left of the sternum and precordial bulge.
4- Excessive flow across the tricuspid valve may produce a third heart sound and short mid-diastolic
murmur at the tricuspid area.

29
5- Excessive blood flow at the pulmonary valve may produce pulsations, dullness and an ejection
systolic murmur in the pulmonary area.
6- The specific auscultatory sign of atrial septal defect is wide fixed splitting of the second heart at the
pulmonary area. The pulmonary component of the second sound is delayed because the right ventricle
takes a long time o empty the excessive volume of blood it receives. The splitting dose not vary with
respiration because: although inspiration causes increase in venous return, yet the resulting rise in right
a trial pressure causes proportionate decrease in the left to right shunt so that the right ventricular
output is constant and the time relation between aortic and pulmonary components of the second sound
remains constant.
7- Progressive pulmonary hypertension occurs in big defects and result in Eisenmenger syndrome. At
this stage the clinical picture consists of: Central cyanosis, signs of pulmonary hypertension, and signs
or right ventricular hypertrophy.
X-RAY PICTURE:
1- Plethoric lung fields. 2- Dilatation of the right atrium, right ventricle and pulmonary artery. 3-
Marked pulsation of the pulmonary artery and its branches seen during screening (hilar dance).
ELECTROCARDIOGRAPHIC FEATURES: The characteristic sign is incomplete right bundle branch
block with rSr' pattern in V1 lead. Signs of right ventricular hypertrophy also appear when pulmonary
hypertension develops. Atrial fibrillation occurs in late cases.
ECHOCARDIOGRAPHY WITH DOPPLER: Must be done for every patient with suspected
congenital heart disease. In A.S.D. it shows the septal defect and dilated right ventricle and abnormal
movement of the interventricular septum characteristic of volume overload on the right ventricle.
Cardiac catheterization may be done in some cases.
COMPLICATIONS:
1- Pulmonary hypertension and reversal of shunt.
2- Right ventricular failure. 3- A trial fibrillation.
TREATMENT: Small defects can be left alone. Large defects should be closed surgically or by
percutaneous insertion of occluder (device that occludes the ASD) .
VENTRICULAR SEPTAL DEFECT
1- In the presence of a defect in the septum, the right ventricle receives both the normal venous and the
shunted blood. If the defect is big right ventricular hypertrophy occurs.

30
2- This excessive blood flows in the pulmonary artery and the pulmonary circulation and then returns
to the left atrium and the left ventricle. This causes: Dilatation of the pulmonary artery, pulmonary
plethora, dilatation of the left atrium, dilatation and hypertrophy of the left ventricle.
3- If the shunt is very big excessive flow may cause heart failure in infancy.
4- If the shunt is large the pulmonary vessels react by vasoconstriction causing pulmonary hypertension
and reversal of shunt (Eisenmenger syndrome).
5- Small V.S.D. does not cause pulmonary hypertension and may close spontaneously. Clinically, the
murmur is very loud (Roger’s disease).
CLINICAL PICTURE: The specific signs of V.S.D. are: 1- A characteristic pansystolic murmur best
heart in the third and fourth left intercostal spaces just lateral to the sternum, usually accompanied by a
thrill. 2- With large shunts the increased flow across the mitral valve may cause a third sound and a
mid-diastolic flow murmur at the apex.
The clinical course depends upon the size of the defect:
1- Small ventricular septal defect: many defects close spontaneously.
2- Moderately large defect:
1st- Progressive pulmonary hypertension and low cardiac output e.g. fatigue, syncope on exercise,
pulsations and palpable loud second heart sound in the pulmonary area, right ventricular hypertrophy,
etc.
2nd- When the pressure in the right ventricle equals that in the left ventricle no blood will flow
across the defect and the murmur diminishes disappears. The patient becomes cyanosed on crying.
3rd- When the shunt is reversed the patient becomes cyanosed.
X-RAY PICTURE: Is normal in cases with small defects. Large defects result in: pulmonary plethora
(overfilled large and tortuous pulmonary arteries), large main pulmonary artery, left and right
ventricular enlargement, left atrial enlargement.
ECHOCARDIOGRAPHY WITH DOPPLER: Can show the size of cardiac chambers. The defect can
sometimes be shown by two-dimensional echo. Color Doppler is very helpful in showing the blood
flow through the defect. Detection of the site of the defect, the magnitude of the shunt and the degree of
pulmonary hypertension can be assessed by this non-invasive method.
CARDIAC CATHETERISATION AND ANGIOGRAPHY: Is done in some cases.
COMPLICATIONS: Infective endocarditis, pulmonary hypertension, and heart failure.
DIFFERENTIAL DIAGNOSIS: A pansystolic murmur at the sternal border can be caused by tricuspid
or mitral incompetence in addition to the ventricular septal defect. Sometimes the murmur of

31
pulmonary stenosis is heard at the third intercostal space but it is usually ejection in type and its
maximal intensity is in the second space. Other causes of systolic murmur at left sternal border are
hypertrophic obstructive cardiomyopathy, subaortic membrane and aortic stenosis.
TREATMENT:
1- To prevent infective endocarditis all patients must receive an antibiotic prophylaxis before
performing minor procedures that may causes bacteremia, e.g. dental extraction, delivery, etc.
2- Small ventricular septal defects should be left alone. Many of them close spontaneously.
3- Surgical closure is indicated if the defect is moderate or large in size, provided that the pulmonary
pressure is normal or moderately elevated. Surgical closure is contraindicated if pulmonary pressure is
severe (Eisenmenger’s syndrome).
PATENT DUCTUS ARTERIOSUS
The ductus arteriosus is normally present in the fetus. It connects the aorta (at the junction of the arch
with the descending aorta) with the pulmonary artery (at the junction of the main pulmonary artery with
its left branch). It normally closes. During the first month after birth:
Effects:
1- The blood flows through the duct from the aorta to the pulmonary artery, i.e. left to right shunt.
2- As the pulmonary artery receives blood both from the shunt and the right ventricle, pulmonary artery
dilatation and pulmonary plethora occur.
3- If the shunt is big pulmonary vasoconstriction and hypertension occurs. When the pressure in the
pulmonary artery equals that of the aorta the shunt will first become confined to the systole only and
then ceases altogether. The murmur, accordingly, will first become only systolic and finally will be
completely inaudible.
5- When the pressure in the pulmonary artery exceeds that of the aorta, the shunt will be reversed and
cyanosis occurs (Eisenmenger’s syndrome).
CLINICAL FEATURES: Patent ductus arteriosus is commoner in females. Its characteristic signs are:
1- A continuous (machinery) murmur that occupies both systole and diastole because the pressure in
the aorta exceeds that of the pulmonary artery all through the cardiac cycle. It is best heard in the first
and second left intercostal spaces. There may be continuous thrill in the same area.
2- With large ductus, the increased flow across the mitral may cause a mid-diastolic murmur.

32
When the pressure in the pulmonary artery exceeds that of the aorta, right to left shunt occurs and
cyanosis appears (Eisenmenger’s syndrome). The deoxygenated blood will flow from the pulmonary
artery across the ductus down the descending aorta. The lower limbs will be cyanosed while the upper
limbs remain pink (differential cyanosis).
X-RAY PICTURE: X-ray is normal in cases with small ductus. In moderate to large ductus the
following signs appear: Pulmonary plethora, enlargement of the left atrium, left ventricle and the aorta.
Hilar dance seen in the hilum by screening.
Differential diagnosis: Other causes of continuous murmur as aorto-pulmonary window, in coarctation
of the aorta, mammary softle, rupture sinus of Valsalva, venous hum...
TREATMENT: Prophylaxis against endocarditis. Closure either surgical or with a device introduced
with percutaneous, transvenous catheter.
CYANOTIC HEART DISEASE
- Tetralogy of Fallot.
- Ebstein anomaly.
- Transposition of the great arteries.
- Total anomalous pulmonary venous drainage.
- Truncus arteriosus.
- Pulmonary arterio-venous malformation.
Acquired cyanotic disease: Eisenmenger Syndrome.
FALLOT’S TETRALOGY

33
PATHOLOGY AND EFFECTS: Fallot’s tetralogy consists of:
1- Severe pulmonary stenosis which causes right ventricular hypertrophy. The pulmonary stenosis is
usually infundibular but sometimes it is both valvular and infundibular.
2- Large ventricular septal defect which makes the pressure equal in both ventricles.
3- The origin of the aorta is abnormally deviated to the right (dextroposed, dextro = right) so that it
lies partly over the right ventricle (the aorta overrides both ventricles).
4- Due to the severe pulmonary stenosis and the large ventricular septal defect, the pressure in both
ventricles is equal. There is rush of blood across the defect and the ventricular septal defect produces
no murmur.
5- Part of the blood pumped by the right ventricle passes in the aorta (right to left shunt) causing
central cyanosis.
In summary Fallot’s tetralogy consists of four components (tetra =4).
1- Pulmonary stenosis.
2- Ventricular septal defect.
3- Dextroposed and overriding aorta.
4- Right ventricular hypertrophy.

34
CLINICAL FEATURES:
1- The patient is cyanosed since birth, (usually after birth by few weeks); the degree of cyanosis
depends on the severity of the pulmonary stenosis.
2- When the patient exercises, cyanosis is increased. In order to increase the blood flow to the head and
brain, the child usually squats to compress the lower limbs against the abdomen and to deviate the
blood from the lower to the upper half of the body. It also increases the systemic arterial resistance. As
the pressure in the aorta rises, more blood will be deviated across the pulmonary stenosis to the lungs.
Thus more oxygenated blood returns to the heart.
3- Chronic cyanosis and tissue anoxia results in: Dyspnea, fatigue, angina, retarded growth,
polycythemia, clubbing of fingers.
4- Sometimes the muscle surrounding the outflow tract of the right ventricle goes into spasm,
especially after excitement and exercise. The blood flow to the lungs decreases markedly and the
oxygenation decreases resulting in attacks of severe cyanosis: cyanotic spells. If prolonged they may
lead to death.
5- The characteristic cardiac signs are:
A- Murmur of pulmonary stenosis (ejection systolic murmur in second left space, usually
accompanied by a thrill.
B- The second heart sound is single and consists only of the aortic component. C- Right
ventricular hypertrophy.
X-RAY PICTURE:
4. Right ventricular hypertrophy causes the apex to be displaced outwards and becomes separated
from the diaphragm.
5. Right-sided aortic arch in some cases.
6. Pulmonary oligemia (the pulmonary artery and its branches are diminished in size due to the
pulmonary stenosis. All the above factors result in a characteristic cardiac shadow: Coeur en sabot
(sabot = wooden shoe).
ELECTROACARDIOGRAPHIC FEATURES: Show moderate right ventricular hypertrophy.
ECHOCARDIOGRAPHY WITH DOPPLER: Delineates the abnormal anatomy. Cardiac
catheterization and angiography is needed for differential diagnosis.
COMPLICATIONS:
1- Polycythemia causes increased viscosity of blood resulting in a tendency towards thrombosis, e.g.
cerebral thrombosis.

35
2- Infective endocarditis
3- Brain abscess results when bacterial emboli are shunted from the venous to the arterial side and
lodge in the brain (paradoxical embolism).
TREATMENT:
1- Surgical correction is indicated in all cases by: Resection of the excessive stenotic infundibular
muscle splitting of the fused pulmonary valve leaflets, and closure of the ventricular septal defect.
2- If he patient is too young, or the condition is too severe, an anastomosis is performed to allow blood
to reach the lungs by: implanting the subclavian artery in the corresponding pulmonary artery (Blalock-
Taussig operation).
3- Cyanotic attacks result from infundibular spasm and constitute an emergency. The are treated by:
Put the patient in the squatting position or compress the flexed lower limbs against the abdomen,
sedation, propranolol (inderal) intravenously. Propranolol is a beta-adrenergic blocker. It depresses the
contractility of the infundibular muscle.
LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION
- Valvular aortic stenosis: 70% of patients with valvular AS a malformation of the valve (usually a
bicuspid valve).
- Discrete subvalvular aortic membrane:
Represents 8-10% of congenital AS. The magnitude of obstruction is variable. Most membranes are
eventually associated with progressive aortic regurgitation and their presence may be an absolute
indication for excision. There is a high recurrence rate after excision (approximately 30% and septal
myotomy is often performed).
COARCTATION OF THE AORTA
Narrowing of the aorta usually just distal to the left subclavian artery. Coarctation may affect other
parts of the aorta or the renal arteries.
EFFECTS:
1- Because of the narrowing, pressure rises in the ascending aorta and the aortic arch and its branches.
This results in hypertension in the upper limbs.
2- Pressure and flow decreases in the descending aorta and its branches producing ischemia in the
abdominal organs and the limbs.
3- Ischemia of the kidneys results in release of renin which raises the blood pressure.

36
4- Hypertension results in left ventricular hypertrophy and it severe results in left ventricular failure.
5- Anastomosis form between the branches of the aorta proximal and distal to the obstruction. The
most important of these connect the subclavian artery through its internal mammary branch to the
intercostal arteries which arise from descending aorta. The intercostal arteries become enlarged and
tortuous and erode the lower border of the ribs causing rib notching. Appreciable anastomosis
develops gradually by time. That is why rib notching is not detectable except after the age of 10. Other
anastomosis develops around the scapula and another connects the superior and inferior epigastric
arteries.
CLINICAL FEATURES:
1- In the majority of cases there are no symptoms and the essential diagnostic feature of coarctation is
that the blood pressure in the upper limbs exceeds that in the lower limbs.
2- The pulse in the upper limbs, neck and suprasternal notch is strong. Pulse in the lower limbs is weak
and delayed or absent.
3- Hypertension in the upper half of the body may produce headache, epistaxis while ischemia of the
lower half may produce thin, underdeveloped lower limbs and claudication in the calf.
4- Visible and palpable pulsations of dilated collateral may be felt in the intercostal areas.
5- A late systolic murmur may be heard on the back due to blood flow in the collaterals. The murmur is
sometimes continuous.
6- The cardiac signs are nonspecific and include: left ventricular hypertrophy, an ejection systolic
murmur heard at the aortic area.
X-RAY PICTURE:
1- Signs of left ventricular hypertrophy.
2- Rib notching is the most specific sign.
ELECTROCARDIOGRAPHIC SIGNS: Left ventricular hypertrophy and strain.
COMPLICATIONS:
1- Hypertension in the upper half of the body may result in: cerebral or subarachnoid hemorrhage, left
ventricular failure, dissection of the aorta.
2- Infective endocarditis.
TREATMENT: surgical resection of the narrowed segment is indicated in moderate and severe cases
preferably during childhood. Balloon dilation with expandable stent is a feasible method of treatment.
All patients must have prophylaxis against endocarditis.

37
PULMONARY STENOSIS
Pulmonary stenosis may be caused by: Congenital fusion of pulmonary valve cusps (congenital
valvular pulmonary stenosis).
EFFECTS:
1- In both valvular and infundibular stenosis the pressure in the right ventricle rises, this causes
hypertrophy of the right ventricle (pressure over-load). Consequently the right atrium hypertrophies.
When the stenosis is severe the output of the right ventricle and the cardiac output are reduced. The
pulmonary blood flow is reduced, i.e. pulmonary oligemia.
CLINICAL FEATURES:
1. Mild cases are as asymptomatic, in severe cases low cardiac output occurs and results in
fatigability, syncope on effort, small volume pulse, cold extremities, etc.
2. An ejection systolic murmur is caused by passage of blood through the stenosed valve. It is best
heard over the pulmonary area. It may be preceded by an ejection click.
3. The pulmonary component of the second heart sound is faint and delayed due to prolonged
contraction of the right ventricle.
4. There is usually a systolic thrill over the pulmonary area.
5. Right ventricular hypertrophy produces a sustained impulse in the third and fourth intercostal
spaces just to the left of the sternum and pulsation in the epigastrium. Forceful right atrial contraction
causes a large wave in the neck veins (the a wave).
X-RAY PICTURE: 1. Pulmonary oligemia occurs in moderate to severe cases and results in reduced
pulmonary vascular markings). 2- Right ventricular enlargement is proportional to the severity of the
stenosis. Right atrial enlargement may also occur. 3. Post-stenosis dilatation of the pulmonary artery
is seen.
ECG FEATURES: Right ventricular hypertrophy.
ECHO FEATURES: Right ventricular hypertrophy, the stenosed pulmonary valve.
TREATMENT: Either percutaneous transvenous balloon dilatation (the standard treatment, first
option) or surgical removal of the valve by open-heart surgery.
Interventions In Congenital Heart Diseases (therapeutic procedures that are used in treatment
without surgery but through catheterization):

38
- Pulmonary stenosis balloon dilatation.
- Aortic stenosis balloon dilatation.
- Coarctation of the aorta balloon dilatation and stent insertion.
- Atrial septal defect insertion of Amplatzer occluder through catheter.
- Patent ductus arteriosus occlusion by insertion of coil.
- Other procedures.
DIAGNOSIS AND MANAGEMENT OF SYNCOPE AND HYPOTENSION
Syncope is a sudden and transient loss of consciousness with associated loss of postural tone. The
occurrence of syncope is 3% in men ad 3.5% in women in the general population. As a general role, the
incidence of syncope increases with age.
Hypotension: When systolic blood pressure (SBP) is less than 90 mmHg or reduction of SBP of 30
mmHg or more from baseline.
Patients with transient episode of altered consciousness (presyncope) and those with complete loss of
consciousness (syncope) are classified into 3 broad categories: cardiac syncope, noncardiac syncope,
and syncope of undetermined etiology. Among all patients with syncope associated with cardiac
disease, sudden cardiac death is extremely high.
Table: Causes of Syncope
Circulatory (reduced cerebral blood flow)
A. Inadequate vasoconstrictor mechanisms
1. Vasovagal (vasodepressor)
2. Postural hypotension
3. Primary autonomic insufficiency
4. Sympathectomy (pharmacologic, due to antihypertensive medications such as methyldopa and
hydralazine, or surgical )
5. Carotid sinus syncope
6. Diseases of the central and peripheral nervous system, including autonomic nerves)
B. Hypovolemia
1. Blood loss – gastrointestinal hemorrhage.
2. Addison’s disease
C. Mechanical reduction of venous return

39
1. Valsalva maneuver. 2. Cough; Micturition.
3. Atrial myxoma, ball valve thrombus.
D. Reduced cardiac output
1. Obstruction to left ventricular outflow: aortic stenosis, hypertrophic subaortic stenosis.
2. Obstruction to pulmonary flow: pulmonary stenosis, primary pulmonary hypertension, pulmonary
embolism.
3. Myocardial: massive myocardial infarction with pump failure.
4. Pericardial: cardiac tamponade
E. Arrhythmias
1. Bradyarrhythmias
a. Atrioventricular (AV) block (second and third degree), with Stokes-Adams attacks
b. Ventricular asystole
c. Sinus bradycardia, sinoatrial block, sinus arrest, sick sinus syndrome
d. Carotid sinus syncope
a. Tachyarrhythmias: Supraventricular tachycardia. Episodic ventricular tachycardia
Other causes of disturbances of consciousness
A. Hypoglycemia
B. Hypoxia
C. Hypoventilation
D. Transient cerebral ischemic attack
E. Emotional disturbances, anxiety attack, hysterical seizures.
Noncardiac Syncope
Neurocardiogenic syncope:
The syndrome of neurocardiogenic syncope, the common faint (also referred to as neurally mediated
hypotension, vasovagal syncope, and vasodepressor syncope), is one of the most common causes of
syncope.
This disorder is due to abnormality in the neuro-cardiovascular interactions responsible for maintaining
systemic and cerebral perfusion.
Diagnostic evaluation:
Head-up tilt (HUT) is essential for the diagnosis of neurocardiogenic syncope. Here we change the
position of the patient from the horizontal to the vertical position. HUT at an angle of 60º to 90º for a
time period of 20 to 60 min is the usual protocol.

40
Management of syncope:
First-line therapy includes counseling the patient to avoid dehydration, prolonged period of standing
motionless, and situations known to trigger syncope. Volume expansion, fludrocortisone may be
helpful in augmenting salt retention and volume expansion.
Alpha-Agonists: Medodrine may prevent neurocardiogenic syncope due to vasoconstrictor effect that
may reduce venous pooling.
Orthostatic Syncope (orthostatic Hypotension):
Orthostatic hypotension is a disorder in which assumption of the upright posture is associated with a
fall in blood pressure. Therapy: is based on treatment of causes.
Management of hypotension: 1- Treatment of the etiology. 2- Avoid dehydration. 3- Medodrine. 4.
Mineralocorticoids as Astonin H.
Cardiac Syncope
It is due to severe diminution of the cardiac output Either due to severe obstructive lesion as tight mitral
stenosis, atrial myxoma, aortic stenosis, obstructive cardiomyopathy or due to arrhythmia whether
tachy or brady. Obstructive lesions and arrhythmias frequently coexist; indeed, one abnormality may
accentuate the other. Common disorders associated with cardiac syncope are listed in table.
Diagnostic evaluation of syncope associated with cardiac disease:
- History & physical examination
- Echocardiography & Doppler
- Standard ECG
- Holter monitor ( 24 h. ECG continuous recording )
- Electrophysiologic study.
- Cardiac catheterization.
Treatment of cardiac syncope: Obstructive Heart Disease, for patients with syncope caused by
obstructive heart disease, cardiac surgery is often the treatment of choice.
Arrhythmic syncope, detailed discussion of therapy for cardiac arrhythmias presented earlier.
Antiarrhythmic drugs, pacemakers and ablation are available tools of management of arrhythmia.
Syncope of undetermined cause: Despite careful diagnostic evaluation, the cause of syncope often
cannot be defined.

41
Sudden Cardiac Death
Definition: Sudden cardiac death describes the unexpected natural death due to cardiac cause within a
short period from the onset of symptoms.
More recent definition focused on time interval of one hour from the symptoms leading to collapse and
then to death.
Incidence: SCD accounts for 300.000 to 400.000 deaths yearly in the United States. SCD is the most
common and often the first manifestation of coronary heart disease (CHD) and is responsible for half
the deaths from cardiovascular disease.
Sudden Cardiac Death in the young: The most common underlying pathological conditions in people
who die of SCD in the first three decades of life are myocarditis, hypertrophic cardiomyopathy,
congenital coronary artery anomalies, atherosclerotic coronary heart disease, conduction system
abnormalities (e.g. long QT), congenital arrhythmogenic disorders, arrhythmias associated with
mitral valve prolapse and aortic dissection. About 40% of SCD in the pediatric population occur in
patients with surgically treated congenital cardiac abnormalities.
Risk factors for Sudden Cardiac Death (SCD):
1- Left ventricular hypertrophy (by ECG)
2- Cholesterol.
3- Hypertension.
4- Cigarette smoking.
5- Diabetes.
6- Alcohol.
7- Obesity.
8- History of coronary heart disease.
9- Age.
10- Positive family history of SCD.
11- Frequent PVCs (Premature ventricular contractions, unsustained ventricular tachycardia).
Cardiac Abnormalities Associated with Sudden Cardiac Death
I. Ischemic heart disease
A) Coronary Atherosclerosis:
- Acute myocardial infarction, - Chronic ischemic cardiomyopathy

42
B) Anomalous origin of coronary arteries.
II. Cardiomyopathies
A. Idiopathic dilated cardiomyopathy
B. Hypertrophic cardiomyopathy
C. Hypertensive cardiomyopathy
D. Arrhythmogenic right ventricular dysplasia
III. Valvular heart disease: Aortic stenosis
IV. Inflammatory and Infiltrative myocardial disease
V. Congenital heart disease.
VI. Primary Electrical Abnormality.
A. Long Q-T syndrome
B. Wolf Parkinson White syndrome (WPW).
C. Idiopathic ventricular tachycardia
D. Idiopathic ventricular fibrillation
E. Brugada syndrome (right bundle block with raised ST in V1 to V3)
VII. Drug and other toxic agents
A. Proarrhythmia (Drug induced arrhythmia)
B. Cocaine and Alcohol. C. Electrolyte abnormalities
Treatment Options for Patients at Risk of Sudden Cardiac Death (SCD)
I. Pharmacologic therapy
1- Beta blockers , Angiotensin-converting enzyme inhibitors
2- Class I antiarrhythmic drugs,
3- Class III antiarrhythmic drugs: Amiodarone, sotalol
II. Device therapy
1- Automatic implantable cardioverter Defibrillator (ICD)
2- External automatic defibrillator
III. Role of surgery: Revascularization
IV. Catheter Ablation therapy.

43
CARDIAC ARRHYTHMIAS
An arrhythmia is any disturbance in the normal sequence of impulse generation and conduction in the
heart.
Anatomy of the conduction system: The conduction system of the heart consists of the sinus node,
internodal tracts, atrioventricular node (AVN), bundle of His, bundle branches (right and left), and
Purkinje fibers.

44
Fig: The pathways of Conduction.
General considerations: Normal cardiac impulses arise from the automatic (pacemaking) cells of the
sinus node and are conducted through the atria to the AV junction then the His-Purkinje system to the
ventricular muscle. Normally the sinus node discharges at a rate of 60-100/min.
Mechanisms of arrhythmias
A- Disturbance of impulse formation: may result from either:
1- Disturbed normal automaticity:
2- Triggered activity: Hyper-excitable focus which discharges ectopic impulses.
B- Disturbance of Impulse conduction: e.g. heart block
Classification of arrhythmia:
Clinical classification:
- Rapid, regular. Sinus tachycardia, supraventricular tachycardia, atrial flutter, ventricular
tachycardia.
- Rapid, irregular. Sinus arrhythmia, multiple ectopic beats whether atrial or ventricular, atrial
fibrillation.
- Slow, regular. Sinus bradycardia, nodal rhythm, complete heart block.
- Slow, irregular. Slow atrial fibrillation.

45
Disturbances in Sinus Rhythm
Sinus tachycardia
Cardiac impulses arise in the sinus node at a rate more than 100/min.
Etiology:
A- Physiological: Infancy, childhood, exercise and excitement.
B- Pharmacological: Sympathomimetic drugs such as epinephrine and isoproterenol.
Parasympatholytic drugs such as atropine. Thyroid hormones, nicotine, caffeine, alcohol.
C- Pathological: Fever, hypotension, heart failure, pulmonary embolism, hyperkinetic circulatory
states as anemia.
Treatment: 1- Treatment of the underlying etiology. 2- Propranolol.
Sinus Bradycardia
Cardiac impulses arise in the sinus node at a rate less than 60/min.
Etiology:
A- Physiologic: Athletes, sleep, and carotid sinus compression.
B- Pharmacologic: Digitalis, propranolol, verapamil and diltiazem.
C- Pathologic: Convalescence from infections, hypothyroidism, obstructive jaundice, rapid rise of the
intracranial tension, hypothermia and myocardial infarction (particularly inferior wall infarction).
Treatment:
1- Treatment of the underlying etiology is usually all that is needed.
2- If the patient is hemodynamically compromised, Atropine 0.6 – 1.0 mg IV may be given and
repeated every 3 hours (maximum 2.5 mg in two hours).
SICK SINUS SYNDROME: This term is applied to a syndrome encompassing a number of sinus
nodal abnormalities that include: 1- persistent spontaneous sinus bradycardia not caused by drugs, and
inappropriate for the physiological circumstance, 2- apparent sinus arrest or exit block, 3- combinations
of SA and AV conduction disturbances, or 4- alternation of paroxysms of rapid and slow atrial and
ventricular rates (bradycardia-tachycardia syndrome).

46
FIG. Normal intracardiac electrograms.
PREMATURE BEATS (EXTRASYSTOLES)
These are cardiac impulses of ectopic origin occurring earlier than expected in the prevailing rhythm.
The ectopic focus may be: 1- Atrial resulting in atrial premature beat. 2- AV junctional (arising from
bundle of His) resulting in AV junctional premature beat. 3- Ventricular resulting in ventricular
premature beat.
Etiology:
A- Physiological: Emotions, exercise and fatigue.
B- Pharmacological: Coffee, alcohol, tobacco, catecholamines, digitalis and hypoxia.
C- Pathological: Various infections, digestive disturbances, hyperthyroidism and all cardiovascular
disorders.
SUPRAVENTRICULAR TACHYARRHYTHMIAS
All tachyarrhythmias that originate above the bifurcation of the bundle of His are classified as
supraventricular arrhythmias (SVT). The atrial rate must be 100 or more beats per minute for a
diagnosis.

47
SVTs may be separated into three groups based on duration: brief paroxysms, persistent, and chronic
(permanent).
Arrhythmias that are paroxysmal in onset and offset (e.g., paroxysmal SVT due to AV nodal reentry or
WPW syndrome, paroxysmal atrial fibrillation, paroxysmal atrial flutter) tend to be recurrent and of
short duration; i.e., seconds to hours.
Persistent tachycardias (e.g., sinus tachycardia, ectopic atrial tachycardia (nonparoxysmal), multifocal
atrial tachycardia, longer episodes of PSVT or atrial flutter or fibrillation) may persist for days or
weeks.
Longstanding or chronic SVTs (chronic atrial flutter, chronic atrial fibrillation) do not revert if
untreated, often fail to revert even with attempted treatment, and if reverted will frequently recur
despite therapy.
Supraventricular tachyarrhythmias include; atrial tachycardia, atrial flutter, atrial fibrillation and AV
tachycardias.
ATRIAL FLUTTER
Atrial flutter is a rapid regular atrial tachyarrhythmia that is less common than the PSVTs or atrial
fibrillation. It is observed in the presence of underlying atrial abnormalities such as those secondary to
mitral valve disease, congenital heart disease, cardiomyopathies, and, less frequently, coronary artery
disease.
Untreated atrial flutter usually has atrial rates between 240 and 340 per minute, commonly very close
to 300 per minute. The ventricular rate in atrial flutter is usually a defined fraction of the atrial rate 2: 1
conduction generating a ventricular rate of 150 per minute and 4:1 conduction at 75 per minute.
Clinically, atrial flutter may occur in brief, persistent, or chronic forms, and therapeutic approaches are
influenced by the clinical pattern.
Electrocardiographic Features
Atrial flutter generates a defined pattern of atrial activity in the ECG. Classically, a saw-tooth pattern is
identifiable in leads II, 111, and aVF. A narrow QRS complex tachycardia at a rate of 150 per minute
should always lead to the consideration of atrial flutter. Carotid sinus massage will not interrupt atrial
flutter but nonetheless may be very helpful in distinguishing flutter from other mechanisms,
impairment of AV nodal conduction causes an abrupt change from a rate of 150 per minute to 75 per
minute or less.

48
Management of atrial flutter: - If the patient is hemodynamically compromised, D.C. cardioversion
using low energies (around 50 joules) should be instituted.
- Administering a Class IA antiarrhythmic agent (i.e., quinidine, procainamide, or disopyramide). IC
antiarrhythmic drugs, flecainide and propafenone, are as effective, if not more effective than Class IA
drugs. Class III antiarrhythmic agents (i.e., amiodarone, sotalol) may also be quite effective. In general,
atrial flutter is difficult to suppress completely with drug therapy. - The ventricular rate is slowed by
digitalis and/or propranolol or verapamil before antiarrhythmics are instituted to avoid very rapid rates
associated with drug induced 1:1 AV conduction.
- At present, catheter ablation provides the best hope of cure.
FIG. A 12-lead ECG of a typical case of type 1 atrial flutter.
FIG. A 12-lead ECG of a typical case of type 1 atrial flutter.

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FIG: Atrial flutter with AV block varying between 2: 1 and 4: 1.
AV Nodal Reentrant Tachycardia
Electrocardiographic Features: Paroxysmal SVT due to AV nodal reentry is characterized by an
abrupt onset and termination and usually has a narrow QRS complex without clearly discernable P
waves. The rate is commonly in the range of 150 to 250 per minute (commonly 180 to 200 bpm in
adults) and with a regular rhythm.
Management of PSVT Due to AV Nodal Reentry
The acute attack: Vagal maneuvers serve as the first line of therapy. Simple procedures to terminate
paroxysmal SVT
- Carotid sinus massage: If effective the rhythm is abruptly stopped; occasionally only moderate
slowing occurs
- Cold water splash on face.
- Performance of Valsalva's maneuver (often effective).
Intravenous adenosine, Ca channel blockers (verapamil), digoxin or B-blockers are the choices for
managing the acute episodes.
Adenosine, 6 mg given intravenously, followed by one or two 6-mg boluses if necessary, is effective
and safe for acute treatment.
A 5-mg bolus of verapamil (isoptin) , followed by one or two additional 5-mg boluses 10 min apart if
the initial dose does not convert the arrhythmia, has been an effective regimen in up to 90 percent of
patients with PSVT due to AV node reentry. Intravenous digoxin, 0.5 mg infused over 10 min and
repeated if necessary may convert the arrhythmia.
DC cardioversion: Consider DC cardioversion before digitalis or a beta blocker is administered.
Radiofrequency catheter ablation: Should be considered early in the management of patients with
symptomatic recurrent episodes of AV node reentry.

50
AV Reentrant Tachycardia
PSVT Due to Accessory Pathways (The Wolff-Parkinson-White Syndrome)
ELECTROCARDIOGRAPHIC RECOGNITION: Three basic features in the ECG of patients with the
usual form of WPW syndrome caused by an AV connection:
(1) Short P-R interval less than 120 msec during sinus rhythm;
(2) QRS complex duration exceeding 120 msec
(3) Slowly rising onset of the QRS in some leads (delta wave).
The most common tachycardia is characterized by a normal QRS, by ventricular rates of 150 to 250
beats/min and by sudden onset and termination.
Termination of the acute episode should be approached as for AV nodal reentry. In many patients,
particularly those with a very rapid ventricular response, electrical cardioversion is the initial treatment
of choice.

51
The Wolff-Parkinson-White Syndrome
ELECTRICAL ABLATION: Ablation of the accessory pathway is advisable for patients with frequent
symptomatic arrhythmias that are not fully controlled by drugs.
Atrial Fibrillation
The arrhythmia is characterized by multiple electric foci in the atrium causing disorganized atrial
depolarizations without effective atrial contraction. Electrical activity of the atrium can be detected on
ECG as small irregular baseline undulations, called f waves, at a rate of 350 to 600 beats/min. The
ventricular response is grossly irregular (irregular irregularity) and is usually between 100 and 160
beats/min.
It is a common arrhythmia, occurring in 5 – 10 % of individuals over 65 years of age. It also occurs in
a paroxysmal form in younger patients.
The hemodynamic consequences of atrial fibrillation are due to two factors:

52
(1) The loss of atrial systole may impair ventricular function in the noncompliant ventricle [e.g., aortic
stenosis, left ventricular hypertrophy (LVH)] or the dilated ventricle with systolic dysfunction, and
(2) A rapid ventricular rate will encroach upon the diastolic filling period of the left ventricle and the
diastolic flow time of the coronary arteries.
(3) The risk of embolism and stroke is a long-term concern of special importance. Atrial fibrillation
may occur in paroxysmal, persistent, and chronic patterns.
Clinical expression of atrial fibrillation:
Definition Duration
- Paroxysmal Minutes/hours
- Short-lasting Seconds --<1 hour
- Long-lasting >1 hour; -- < 48 hours
- Persistent Two days -- weeks
- Permanent (Chronic) Months / years
Table: Causes of atrial fibrillation
With structural heart disease
- Rheumatic mitral valve disease
- Ischemic heart disease
- Hypertension
- Cardiomyopathy: Dilated, Hypertrophic
- Atrial septal defect, - Constrictive pericarditis, Myocarditis
Without structural heart disease
- Alcohol. Thyrotoxicosis
- Acute pericarditis. Pulmonary embolism
- Sick sinus syndrome, Lone atrial fibrillation

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Atrial Fibrillation
Clinical picture
Onset and offset are sudden in paroxysmal cases.
Symptoms: Paroxysmal AF produces symptoms similar to those of supraventricular tachycardia.
Established AF (persisting for more than two weeks) is better tolerated than the paroxysmal variety.
Congestive heart failure may occur if the attack is prolonged, the ventricular rate is very rapid, or the
underlying heart disease is severe.
Signs:
1- Arterial pulse:
a- Rate is usually 100-150/min. Slower rates may be encountered in old age and in patients receiving
digitalis or beta-blockers.
b- Rhythm shows marked (irregular) irregularity. c- Force is irregular. d- Pulsus deficit: The radial
pulse rate is less than the cardiac rate counted at the apex beat. This is due to inability of the week
ventricular contractions following short diastolic periods to open the aortic valve.

54
2- Neck veins show systolic expansion; no “a” waves are seen.
3- Auscultation reveals varying intensity of S1.
4- Exercise increases the pulse irregularity and deficit.
Electrocardiogram: The P waves are replaced by irregular f waves. The QRS complexes are normal in
shape but irregularly spaced.
Complications: 1- Atrial thrombosis due to stagnation of blood in the fibrillating atria. The formed
thrombi may embolize in the systemic and pulmonary circulations. 2- Heart failure due to loss of the
atrial contribution to contractility and the cardiac output.
Atrial fibrillation (AF) progressed to ventricular fibrillation (VF)
Treatment of Atrial Fibrillation
Pharmacologic Management of Patients with Recurrent Persistent or Permanent AF:
- Recurrent Persistent AF:
A) Minimal or no symptoms: Anticoagulation and rate control as needed.
B) Disabling symptoms in AF:
1- Anticoagulation and rate control
2- Antiarrhythmic drug therapy
3- Electrical cardioversion as needed, continue anticoagulation as needed and therapy to maintain
sinus rhythm
- Permanent AF: Anticoagulation and rate control as needed.

55
AF management
Antiarrhythmic Drug Therapy to Maintain Sinus Rhythm in Patients with Recurrent
Paroxysmal or Persistent AF:
A) No or minimal heart disease:
1- Flecainide, propafenone, sotalol
2- Amiodarone, dronedarone, dofetilide, Disopyramide, procainamide, quinidine
3- Consider non-pharmacological options (ablation).
B) Heart disease present:
a- Heart failure: Amiodarone, dofetilide
1- Coronary artery disease: Sotalol, Amiodarone, dofetilide
2- Dronedarone is allowed only in HF class I or II with precaution.
3- Vernakalant I.V. for aute AF of less than 7 days duration, with many precautions and
contraindications.
C) Hypertension: With
1- With LVH (septum greater than or equal to 1.4 cm): Amiodarone
2- Without this degree of LVH: - Flecainide, propafenone.

56
Drugs for Pharmacologic Cardioversion of AF (Rhythm
control)
Drug Route of Admin. And Dosage
Amiodarone Oral: 1.2 to 1.8 g /day then 200 to 400 mg /d maintenance.
IV: 1.2 g /d IV continuous or in divided doses, then 200 to 400
mg /d maintenance
Dofetilide Oral: Creatinine clearance > 60 ml/min: 500 mcg BID
Flecainide Oral 200 to 300 mg
IV: 1.5 to 3 mg /kg over 10 to 20 min
Propafenone Oral: 450 to 600 mg
IV: 1.5 to 2 mg per kg over 10 to 20 min
Orally Administered Pharmacological Agents for Heart
Rate Control in Patients with AF
Drug Maintenance dose
Digoxin 0.125 to 0.375 mg daily
Metoprolol* 25 to 100 BID
Propranolol 80 to 360 mg daily in divided doses
Verapamil 120 to 360 mg daily in divided doses
Diltiazem 120 to 360 mg daily in divided doses
Anticoagulation of Patients with Atrial Fibrillation: Indications
Rheumatic mitral valve disease with recurrent or chronic atrial fibrillation.
Dilated cardiomyopathy with recurrent persistent or chronic atrial fibrillation.
Prosthetic valves.

57
Prior to (>3 weeks) elective cardioversion of persistent or chronic atrial fibrillation, and also for 3
weeks after cardioversion (because of atrial stunning).
Coronary heart disease or hypertensive heart disease with recurrent persistent or chronic atrial
fibrillation
Atrial fibrillation in thyrotoxicosis (while awaiting long-term control; elective cardioversion)
Chronic or persistent lone atrial fibrillation, age >60 years
Controversial; or limited data
Coronary or hypertensive heart disease with normal left atrial size, after first episode of paroxysmal
atrial fibrillation
Elective cardioversion of atrial fibrillation of short duration (2-3 days) with normal left atrial size
Chronic or persistent lone atrial fibrillation, age <60 years
Not indicated
Lone atrial fibrillation, short paroxysms (<48 h)
Most clinical settings associated with short paroxysms (minutes to hours)
Relative contraindications
Difficulty controlling prothrombin times. Dementia
Malignancies, especially associated with bleeding risk
Prior major bleeding events. Uncontrolled hypertension
Treatment of Cardiac Arrhythmias with Catheter Ablative Techniques
Radiofrequency ablation destroys tissue by controlled heat production. Catheter ablation is used to treat
patients with four major tachyarrhythmias: atrial flutter/fibrillation, AV nodal reentry, accessory
pathways and ventricular tachycardia.
VENTRICULAR TACHYCARDIA
Specific Forms of Ventricular Tachycardia
Duration: Salvo (3-5 impulses)
Nonsustained VT: (6 impulses, up to 29 seconds)
Sustained VT: (>30 seconds)
The electrocardiographic diagnosis of ventricular tachycardia is suggested by the occurrence of a series
of three or more bizarrely shaped premature ventricular complexes whose duration exceeds 120 msec,
with the ST-T pointing opposite to the major QRS deflection.

58
The rates range from 70 to 250 beats/min. Ventricular tachycardia can be sustained, defined arbitrarily
as lasting longer than 30 sec or requiring termination because of hemodynamic collapse, or
nonsustained (Unsustained), when it stops spontaneously in less than 30 sec.
Ventricular tachycardia (Wide QRS tachycardia)
Management: Intravenous lidocaine or amiodarone, followed by an infusion of the successful drug. If
the arrhythmia does not respond to medical therapy, electrical DC cardioversion can be employed.
Ventricular tachycardia in a patient with right ventricular dysplasia.
CONGENITAL LONG QT INTERVAL SYNDROME

59
The normal QT interval is .43 sec. The congenital long QT interval syndrome, which is present
persistently from childhood, is characterized by the presence of long QT intervals on the standard 12-
lead ECG. The affected patients are prone to episodes of torsade de pointes (ventricular tachycardia
with special polymorphic configuration), which may cause transient light-headedness or syncope or
sudden cardiac death. Arrhythmias may occur at rest, under emotional stress, or with exercise.
ACQUIRED LONG QT INTERVAL SYNDROME
Causes: Antiarrhythmic drugs as quinidine. There is a growing list of other drugs that may prolong the
QT interval, and establish susceptibility to torsade de pointes. These include the phenothiazines, certain
antibiotics, pentamidine, cocaine, and terfenadine, among others.
Management of Congenital Long QT Interval Syndrome: Long-term therapy includes B-adrenergic
blockade. Placement of an ICD should be considered for patients with resistant arrhythmias.
CARDIOVERSION AND DEFIBRILLATION
Differences between cardioversion and defibrillation:
Cardioversion Defibrillation
Elective Emergency
Synchronized Non-synchronized
For AF, A. flutter, SVT, VT For V. fibrillation
50, 100, 150, 200 Joules Start by 200 Joules
Need sedative first Patient is unconscious
VENTRICULAR FLUTTER AND FIBRILLATION
MANAGEMENT: Immediate nonsynchronized DC electrical shock using 200 to 360 joules is
mandatory treatment for ventricular fibrillation. Cardiopulmonary resuscitation is employed only until
defibrillation equipment is ready. Time should not be wasted with cardiopulmonary resuscitation
maneuvers if electrical defibrillation can be done promptly.
The Implantable Cardioverter Defibrillator (ICD)
Apparatus (pacemaker) that gives electric shock if the patient develops ventricular fibrillation. The
pacemaker is inserted in the sub-pectoral area.
ICD indications

60
A. Cardiac arrest not due to acute ischemia or infarction or reversible causes.
B. Documented sustained VT with hemodynamic compromise.
C. Syncope of unknown origin in structural heart disease patients with inducible sustained VT.
D. Cardiomyopathy ischemic or non-ischemic with ejection fraction 30% or lower (MADIT II results).
AV HEART BLOCK
Heart block is a disturbance of impulse conduction that can be permanent or transient, owing to
anatomical or functional impairment.
The conduction disturbance is classified by severity in three categories.
During first degree heart block, conduction time is prolonged but all impulses are conducted (P-R
interval > 0.2 sec.).
Second degree heart block occurs in three forms:
Mobitz type I (Wenckebach) and type II; and persistent 2:1 block.
Mobitz Type I heart block is characterized by a progressive lengthening of the conduction time until an
impulse is not conducted (Fig).
Mobitz Type II heart block denotes occasional (Mobitz II) or repetitive sudden block of conduction of
an impulse without prior measurable lengthening of conduction time. When no impulses are conducted,
complete or third degree block is present.
Mobitz type I (Wenckebach) block
Mobitz Type II second degree heart block
COMPLETE AV BLOCK

61
ELECTROCARDIOGRAPHIC RECOGNITION: Complete AV block occurs when no atrial activity
conducts to the ventricles and therefore the atria and ventricles are controlled by independent
pacemakers. Thus, complete AV block is one type of complete AV dissociation.
The ventricular focus is usually located just below the region of block, which can be above or below
the His bundle bifurcation. The ventricular rate of acquired complete heart block is less than 40
beats/min but may be faster in congenital complete AV block.
CLINICAL FEATURES. Block proximal to the His bundle generally exhibits normal QRS complexes
and rates of 40-60 beats/min because the escape focus that controls the ventricle arises in or near the
His bundle.
Causes: Surgery, electrolyte disturbances, endocarditis, tumors, Chagas' disease, rheumatoid nodules,
calcific aortic stenosis, myxedema, polymyositis, infiltrative processes (such as amyloid, sarcoid, or
scleroderma). In the adult, drug toxicity, coronary disease, and degenerative processes appear to be the
most common causes of AV heart block.
COMPLETE AV BLOCK
MANAGEMENT: Temporary or permanent pacemaker insertion is indicated in patients with
symptomatic bradyarrhythmias. Vagolytic agents such as atropine (novatropine 15 drops every 8 hours)
are useful, while catecholamines such as isoproterenol (Allupent syrup 5 ml every 8 hours) can be used
transiently to treat patients who have heart block. The use of transcutaneous pacing is preferable.
ELECTROPHYSIOLOGIC STUDY
EP study is an invasive procedure in which intracardiac electrode catheters are used to evaluate cardiac
arrhythmias and to select various therapeutic options.
Indications of EPS:
Diagnostic:
 Aborted SCD (sudden cardiac death). - Syncope of undetermined cause.
 Recurrent WCT (wide complex tachycardia). - Ventricular tachycardia.
 Recurrent tachycardia with WPW syndrome.
 Symptomatic refractory NCT (narrow complex tachycardia).

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Therapeutic:
 Catheter ablation for AVNRT (AV nodal reentrant tachycardia), WPW (Wolff Parkinson White
syndrome), VT (Ventricular Tachycardia), Atrial fibrillation.
 Acute termination of hemodynamically unstable tachycardias.
CARDIAC PACEMAKERS
Cardiac pacemakers are devices either implanted permanently or inserted temporarily, consisting of a
pulse generator and an electrode catheter that is placed transvenously into the right ventricle and/or
atrium. Small electrical impulses, generated by the pulse generator and delivered via the electrode
catheter depolarize the heart. Pacemakers are widely used for treating bradyarrhythmias but can also be
useful for treatment of some tachyarrhythmias.
Temporary pacing is indicated for symptomatic second or third degree heart block caused by transient
drug intoxication or electrolyte imbalance in the setting of an acute MI, CHB, or Mobitz II second
degree AV Block. Symptomatic sinus bradycardia, AF with a slow ventricular response.
Indications for permanent pacemaker implantation:
Symptomatic bradycardia, due to either sinus node dysfunction or AV nodal block, in absence of a
reversible cause, constitutes a class I indications for permanent pacing. Asymptomatic conditions that
are also considered class I indications for permanent pacing include:
1- 3rd
degree AV Block .
2- Persistent advanced 2nd
degree or 3rd
degree AVB after acute MI with demonstrated block in His-
Purkinje system (BBB).
3- Chronic bifascicular or trifascicular block with intermittent type II second or third degree AV
Block.
Pacing modalities: a four-letter alphabetic code is used to identify pacing modalities. The first initial
defines the chamber that is paced (V: ventricle, A: atrium, D: dual chamber). The second identifies the
chamber that is sensed (V, A, D), the third indicates the response to sensed event (I: inhibited, T:
triggered, D: dual function), and the fourth when present, denotes, R: rate responsive node. VVI &
DDD modes are used most commonly. VVI units pace and sense ventricle and a sensed (native) event
inhibits the ventricular stimulus. DDD units, pace and sense both chambers, events sensed in the
atrium inhibit the atrial stimulus and trigger a ventricular response after an appropriate interval,
where as ventricle-sensed events inhibit ventricular and atrial outputs.

63
Antiarrhythmic Drugs
Class Mode of
Action
Drugs Indication Dose Side Effects
Class
IA
Reduces
rate of
entry of
sodium
into the
cell
Quinidine
(Quinidine)
For
supraventricular
and ventricular
arrhythmias
including
conversion of AF or
A flutter, SVT, VT
600 – 1000
mg/day
Prolongation of QT
interval, risk of
Torsade de pointes.
Quinidine syncope,
quinidine induced
sudden death.
Diarrhea, vomiting
Procainamide
(Pronestyl)
Is effective
against
supraventricular
and ventricular
arrhythmias
2-6
mg/min
IV. 350-
1000 mg q
6 h PO
SLE like
syndrome,
prolonged QT,
nausea, rash,
myalgia,
Disopyramid
e (Norpace)
Is effective
against
supraventricular
and ventricular
arrhythmias
100-400
mg q 8 h
Worsening of
heart failure,
anticholinergic
actions as urine
retention. Avoid
in pts with
glaucoma
Class
IB
Lidocaine
(Zylocain)
Ventricular
arrhythmias only
1-4 mg/min
IV (50-150
mg IV
loading
dose)
Confusion,
convulsions
Mexiletine
(Mexitil)
Ventricular
arrhythmias only
150-300
mg q 6-8 h
Confusion,
tremor,
bradycardia,
hypotension
Class
IC
Flecainide
(Tambocor)
Is very effective
for ventricular
and
supraventricular
tachycardias
100-200
mg q 12 h
PO
Aggravation of
arrhythmia
(proarrhythmia),
negative inotropic
effect, depression
of sinus node

Principles of cardiology total.samir rafla

Principles of cardiology total.samir rafla

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