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Test #6 Q.Id = 588 Question 17 Of 46 Division : Cardiology

An 11-year-old male is brought to the emergency room after he collapsed in

a school athletic event. ECG shows pulseless ventricular tachycardia.

Attempts at cardioversion fail and he dies in spite of all resuscitative efforts.

Which of the following conditions is the most likely cause of sudden cardiac

death in this young athlete?

A. Hypertrophic cardiomyopathy

B. Congenital anomaly of coronary arteries

C. Arrhythmogenic right ventricular dysplasia

D. Complex atrial septal defect

E. Ebstein’s anomaly

F. Congenital Brugada syndrome

G. Ruptured aortic aneurysm

Explanation:

Hypertrophic cardiomyopathy is the most common cause of sudden cardiac

death (SCD) in young athletes. There are many potential mechanisms of

sudden cardiac death in HCM. The most common are ventricular

tachyarrhythmias. Another proposed mechanism is myocardial ischemia,

due to high demand.

Congenital anomalies of coronary arteries, arrhythmogenic right ventricular

dysplasia, atrial septal defect, and Ebstein’s anomaly are all important

causes of SCD in young athletes but they are far less frequent than

hypertrophic cardiomyopathy.

Educational Objective:

Hypertrophic cardiomyopathy is the most common cause of sudden cardiac

death in young athletes.

68% of people answered this question correctly

file:///H|/Documents and Settings/XPPRESP3/Local Settings/T...Rar$EX00.461/Test6_Car_Done/Question 1 of 46_Cardiology.htm14/04/1429 04:08:45 •

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Test #6 Q.Id = 594 Question 18 Of 46 Division : Cardiology

A 60-year-old female presents with dyspnea and ankle edema. Her dyspnea

was mild 6 months ago and used to occur with moderate exertion but now

has progressed to dyspnea, even at rest. She denies any chest pain,

syncope, or palpitations. She does not smoke or drink alcohol. She denies

diabetes mellitus, hypertension, or hyperlipidemia. Her vitals are, PR: 80/

min; BP: 130/70 mmHg; Temperature: 37.1C(98.9); RR: 14/min.

Examination is significant for jugular venous distension, bilateral ankle

edema, and tender hepatomegaly. Kussmaul’s sign is positive and pulsus

paradoxus is negative. Chest auscultation shows bibasilar crackles. Heart

sounds are distant and there is no murmur. Chest x-ray shows mild

cardiomegaly and a right-sided pleural effusion. EKG shows low voltage

QRS complexes and nonspecific ST-T wave changes. Echocardiography

shows symmetrical thickening of left ventricular walls, normal ventricular

size, and slightly reduced systolic function. CT scan of the chest shows

normal pericardial thickness. Based on these findings, which of the following

is the most likely diagnosis in this patient?

A. Dilated cardiomyopathy

B. Restrictive cardiomyopathy

C. Hypertrophic cardiomyopathy

D. Cardiac tamponade

E. Constrictive pericarditis

Explanation:

Restrictive cardiomyopathy is characterized by severe diastolic dysfunction

due to a stiff ventricular wall. Chest x-ray shows only mild enlargement of

the cardiac silhouette. Echocardiography usually shows a symmetrically

thickened ventricle wall, normal or slightly reduced left ventricle size and

normal or near normal systolic function. Kussmaul’s sign may also be

present. The apical impulse is easily palpable in restrictive cardiomyopathy

as opposed to constrictive pericarditis. Restrictive cardiomyopathy is difficult

to differentiate from constrictive pericarditis. With constrictive pericarditis,

chest x-rays may show pericardial calcifications and the CT scan usually

shows increased thickness of pericardium. Kussmaul’s sign may be positive

in both conditions. With constrictive pericarditis, the thickness of

myocardium will be normal.

file:///H|/Documents and Settings/XPPRESP3/Local Setting...$EX00.284/Test6_Car_Done/Question 1 of 46_Cardiology.htm (1 of 2)19/04/1429 01:33:55 •

Normal Labs

Question 18 of 46

Dilated cardiomyopathy is characterized by impaired systolic function of left

and right ventricle leading to progressive cardiac enlargement. Chest x-ray

shows marked or moderate enlargement of cardiac silhouette.

Echocardiography shows systolic dysfunction and left ventricular dilatation

with normal thickness of the ventricular wall.

Hypertrophic cardiomyopathy is characterized by asymmetric left ventricular

hypertrophy. In HCM, a harsh systolic murmur best heard at the left sternal

border is also present. Chest x-ray shows mild enlargement of cardiac

silhouette. Echocardiography shows vigorous systolic function, asymmetric

septal hypertrophy and in some cases systolic anterior motion of the mitral

valve. Due to the hypertrophy of the left ventricular wall, there is diastolic

dysfunction.

In cardiac tamponade, there is sinus tachycardia and hypotension. Pulsus

paradoxus is also present in this condition. There is jugular venous

distension with a prominent 'y' descent.

Educational Objective:

Know how to diagnose restrictive cardiomyopathy and how to differentiate it

from other cardiac conditions that may present similarly

.

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