[MCQ] Hypertrophic cardiomyopathy (HCM)

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Hypertrophic cardiomyopathy (HCM)

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Question 1

In HCM, ubiquitous pathophysiologic abnormality is ?

A	Systolic dysfunction
B	Diastolic dysfunction
C	Systolic + diastolic dysfunction
D	None of the above

Question 1 Explanation:

Ubiquitous pathophysiologic abnormality in HCM is diastolic dysfunction, characterized by increased stiffness of hypertrophied muscle. This results in elevated diastolic filling pressures & is present despite of a hyperdynamic left ventricle

Question 2

In HCM, most common mutations occur in which of the following genes ?

A	Cardiac alpha-myosin heavy chain gene
B	Cardiac beta-myosin heavy chain gene
C	Cardiac myosin light chain gene
D	Cardiac troponins C, I, and T gene

Question 2 Explanation:

In HCM, most common are mutations of the cardiac beta-myosin heavy chain gene. Others involve alpha-myosin heavy chains, cardiac troponins C, I, and T, cardiac myosin-binding protein C, actin, myosin light chains & titin

Question 3

In HCM, most common mutations of cardiac beta-myosin heavy chain gene is on ?

A	Chromosome 10
B	Chromosome 12
C	Chromosome 14
D	Chromosome 16

Question 3 Explanation:

In HCM, most common mutations of cardiac beta-myosin heavy chain gene is on chromosome 14

Question 4

At what age, full genetic expression occurs in first-degree relatives of patients with familial HCM ?

A	5 years
B	10 years
C	20 years
D	30 years

Question 4 Explanation:

Echocardiographic studies have confirmed that by age 20 years, full genetic expression occurs in about half of the first-degree relatives of patients with familial HCM.

Question 5

Which of the following is the most common cause of SCD in young competitive athletes ?

A	MVP
B	Long QT syndrome
C	HCM
D	Tetralogy of Fallot

Question 5 Explanation:

HCM is the most common cause of SCD in young competitive athletes.

Question 6

Which of the following is the most common cause of sudden death among young athletes ?

A	Mitral valve prolapse syndrome
B	Hypertrophic cardiomyopathy
C	Thyrotoxicosis
D	Anxiety

Question 6 Explanation:

Hypertrophic cardiomyopathy is the most common cause of sudden death among young athletes

Question 7

What proportion of first-degree relatives of patients with familial HCM have evidence of the disease ?

A	One-fourth
B	One-third
C	One-half
D	One-half

Question 7 Explanation:

Echocardiographic studies have confirmed that about one-third of the first-degree relatives of patients with familial HCM have evidence of disease.

Question 8

In symptomatic HCM, the most common complaint is ?

A	Dyspnea
B	Palpitation
C	Angina pectoris
D	Syncope

Question 8 Explanation:

In symptomatic HCM patients, most common complaint is dyspnea, due to diastolic ventricular dysfunction which impairs ventricular filling & leads to elevated LV diastolic, left atrial & pulmonary capillary pressures. Other symptoms include syncope, angina pectoris & fatigue. Symptoms are not closely related to the presence or severity of an outflow pressure gradient

Question 9

Mendelian transmission of single-gene defects may occur in ?

A	Hypertrophic cardiomyopathy
B	Marfan syndrome
C	Long QT syndrome
D	All of the above

Question 9 Explanation:

Familial clustering due to Mendelian transmission of single-gene defects may occur in hypertrophic cardiomyopathy, Marfan syndrome & sudden death associated with a prolonged QT syndrome. Essential hypertension or coronary atherosclerosis are often polygenic disorders

Question 10

Risk of sudden death in HCM correlates with all except ?

A	Ventricular tachycardia on ambulatory monitoring
B	Marked ventricular septal hypertrophy (>30 mm)
C	Abnormal blood pressure response to exercise
D	Severity of symptoms

Question 10 Explanation:

HCM patients at higher risk of sudden death are those with a history of resuscitation from sudden cardiac death, ventricular tachycardia on ambulatory monitoring or at electrophysiologic testing, marked ventricular hypertrophy (ventricular septal thickness >30 mm), syncope (in children), genetic mutations associated with an increased risk, abnormal blood pressure response to exercise & a family history of sudden death. There is no correlation between the risk of sudden death and the severity of symptoms, but there is an increased risk of death in patients with outflow gradients

Question 11

Systolic murmur in obstructive HCM is best heard at ?

A	A1 area
B	A2 area
C	Pulmonary area
D	Lower left sternal border

Question 11 Explanation:

Hallmark of obstructive HCM is a systolic murmur which is typically harsh, diamond-shaped & begins after S1, since ejection is unimpeded early in systole. Murmur is best heard at lower left sternal border as well as at apex, where it is often more holosystolic & blowing in quality due to mitral regurgitation that usually accompanies obstructive HCM.

Question 12

Which of the following accompanies obstructive HCM ?

A	AS
B	AR
C	MS
D	MR

Question 13

Maneuvers that increase the murmur of obstructive HCM include all except ?

A	Valsalva maneuver
B	Tachycardia
C	Sudden standing
D	Passive leg raising

Question 14

Maneuvers that decrease the murmur of obstructive HCM include all except ?

A	Squatting
B	Sustained handgrip
C	Nitroglycerin
D	Passive leg raising

Question 14 Explanation:

Interventions that increase myocardial contractility like exercise, sympathomimetic amines & digitalis glycosides, and those that reduce ventricular volume like Valsalva maneuver, sudden standing, nitroglycerin, amyl nitrite or tachycardia, may all cause an increase in the gradient & the murmur. Conversely, elevation of arterial pressure by phenylephrine, squatting, sustained handgrip, augmentation of venous return by passive leg raising & expansion of the blood volume all increase ventricular volume & ameliorate the gradient & murmur.

Question 15

Left ventricular cavity in HCM is typically ?

A	Small
B	Normal
C	Large
D	Any of the above

Question 15 Explanation:

Any of the above

Question 16

In echocardiography of HCM, interventricular septum is how many times thicker than high posterior left ventricular free wall ?

A	0.5 times
B	0.8 times
C	1.1 times
D	1.3 times

Question 16 Explanation:

HCM echocardiogram, demonstrates LVH, with septum 1.3 or more times the thickness of high posterior left ventricular free wall

Question 17

Which of the following investigation is best for accurate measurements of regional hypertrophy and in identifying sites of regional fibrosis ?

A	ECG
B	Echocardiography
C	CMRI
D	LV Angiography

Question 17 Explanation:

CMRI is superior to echocardiography in providing accurate measurements of regional hypertrophy and in identifying sites of regional fibrosis.

Question 18

Complications of HCM include ?

A	Atrial fibrillation
B	Infective endocarditis
C	Sudden death
D	All of the above

Question 18 Explanation:

All of the above

Question 19

Which of the following calcium channel blockers should be avoided in cases of obstructive HCM ?

A	Verapamil
B	Diltiazem
C	Nifedipine
D	All of the above

Question 19 Explanation:

Verapamil & diltiazem reduces stiffness of ventricle, reduces elevated diastolic pressures, increase exercise tolerance & reduces severity of outflow tract pressure gradients. Nifedipine should be avoided.

Question 20

Which of the following drugs should be avoided in cases of obstructive HCM ?

A	Digitalis
B	Diuretics
C	Nitrates
D	All of the above

Question 20 Explanation:

Digitalis, diuretics, nitrates, vasodilators are best avoided, particularly in patients with known left ventricular outflow tract pressure gradients.

Question 21

Which out of the following drugs should not be used in a case of obstrutive HCM ?

A	Beta-blockers
B	Digitalis
C	Nondihydropyridine calcium blockers
D	Disopyramide

Question 21 Explanation:

Beta blockers block the effects of catecholamines that exacerbate outflow tract obstruction & to slow the heart rate so that diastolic filling is enhanced. Disopyramide exerts negative inotropic effects that decreases the outflow gradient and thereby improve symptoms. Digitalis increases left ventricular outflow tract pressure gradients by increasing myocardial contractility.

Question 22

Which out of the following drugs should not be used in a case of obstrutive HCM ?

A	Beta-blockers
B	Digitalis
C	Nondihydropyridine calcium blockers
D	Disopyramide

Question 22 Explanation:

Question 23

Which of the following drug is protective against SCD in HCM ?

A	Amiodarone
B	Amiodarone
C	Disopyramide
D	Diltiazem

Question 23 Explanation:

Amiodarone is effective in reducing the frequency of supraventricular as well as life-threatening ventricular arrhythmias and anecdotal data suggest that it may reduce the risk of SCD

Question 24

Which of the following is false about HCM ?

A	Found in ~1 in 500 of general population
B	Systolic anterior motion of mitral valve is characteristic
C	Myocardial perfusion defects on thallium 201 radionuclide scintigraphy frequent
D	None of the above

Question 24 Explanation:

HCM is found in ~1 in 500 of the general population. Dynamic LV subaortic outflow tract pressure gradient due to midsystolic proximity of anterior mitral valve leaflet against hypertrophied septum is typical of obstructive HCM. Radionuclide scintigraphy with thallium 201 frequently shows myocardial perfusion defects even in asymptomatic patients.

Question 25

What is meant by “burnt out HCM” ?

A	HCM not manifesting in offsprings
B	HCM disappearing with advancing age
C	HCM progressing to DCM with disappearance of preexisting outflow pressure gradient
D	HCM with AR

Question 25 Explanation:

Progression of HCM to left ventricular dilatation & dysfunction (DCM) with wall thinning & disappearance of a preexisting outflow pressure gradient is called burnt out HCM. It occurs in 5 - 10% of patients & may be associated with nonresponsive CHF requiring cardiac transplantation

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See all quizzes of the Hypertrophic cardiomyopathy at here:

1. In HCM, ubiquitous pathophysiologic abnormality is ?
A. Systolic dysfunction
B. Diastolic dysfunction
C. Systolic + diastolic dysfunction
D. None of the above

2. In HCM, most common mutations occur in which of the following genes ?
A. Cardiac alpha-myosin heavy chain gene
B. Cardiac beta-myosin heavy chain gene
C. Cardiac myosin light chain gene
D. Cardiac troponins C, I, and T gene

3. In HCM, most common mutations of cardiac beta-myosin heavy chain gene is on ?
A. Chromosome 10
B. Chromosome 12
C. Chromosome 14
D. Chromosome 16

4. At what age, full genetic expression occurs in first-degree relatives of patients with familial HCM ?
A. 5 years
B. 10 years
C. 20 years
D. 30 years

5. Which of the following is the most common cause of SCD in young competitive athletes ?
A. MVP
B. Long QT syndrome
C. HCM

D. Tetralogy of Fallot

6. Which of the following is the most common cause of sudden death among young athletes ?
A. Mitral valve prolapse syndrome
B. Hypertrophic cardiomyopathy
C. Thyrotoxicosis
D. Anxiety

7. What proportion of first-degree relatives of patients with familial HCM have evidence of the disease ?
A. One-fourth
B. One-third
C. One-half
D. Three-fourth

8. In symptomatic HCM, the most common complaint is ?
A. Dyspnea
B. Palpitation
C. Angina pectoris
D. Syncope

9. Mendelian transmission of single-gene defects may occur in ?
A. Hypertrophic cardiomyopathy
B. Marfan syndrome
C. Long QT syndrome
D. All of the above

10. Risk of sudden death in HCM correlates with all except ?
A. Ventricular tachycardia on ambulatory monitoring
B. Marked ventricular septal hypertrophy (>30 mm)
C. Abnormal blood pressure response to exercise
D. Severity of symptoms

11. Systolic murmur in obstructive HCM is best heard at ?

A. A1 area
B. A2 area
C. Pulmonary area
D. Lower left sternal border

12. Which of the following accompanies obstructive HCM ?
A. AS
B. AR
C. MS
D. MR
13. Maneuvers that increase the murmur of obstructive HCM include all except ?
A. Valsalva maneuver
B. Tachycardia
C. Sudden standing
D. Passive leg raising
14. Maneuvers that decrease the murmur of obstructive HCM include all except ?
A. Squatting
B. Sustained handgrip
C. Nitroglycerin
D. Passive leg raising

15. Left ventricular cavity in HCM is typically ?
A. Small
B. Normal
C. Large
D. Any of the above

16. In echocardiography of HCM, interventricular septum is how many times thicker than high posterior left ventricular free wall ?
A. 0.5 times
B. 0.8 times
C. 1.1 times
D. 1.3 times

17. Which of the following investigation is best for accurate measurements of regional hypertrophy and in identifying sites of regional fibrosis ?
A. ECG
B. Echocardiography
C. CMRI
D. LV Angiography

18. Complications of HCM include ?
A. Atrial fibrillation
B. Infective endocarditis
C. Sudden death
D. All of the above

19. Which of the following calcium channel blockers should be avoided in cases of obstructive HCM ?
A. Verapamil
B. Diltiazem
C. Nifedipine
D. All of the above

20. Which of the following drugs should be avoided in cases of obstructive HCM ?
A. Digitalis
B. Diuretics
C. Nitrates
D. All of the above

21. Which out of the following drugs should not be used in a case of obstrutive HCM ?
A. Beta-blockers
B. Digitalis
C. Nondihydropyridine calcium blockers
D. Disopyramide

22. Which of the following drug is protective against SCD in HCM ?
A. Amiodarone
B. Beta-adrenergic blockers

C. Disopyramide
D. Diltiazem

23. Which of the following is false about HCM ?
A. Found in ~1 in 500 of general population
B. Systolic anterior motion of mitral valve is characteristic
C. Myocardial perfusion defects on thallium 201 radionuclide
scintigraphy frequent
D. None of the above

24. What is meant by “burnt out HCM” ?
A. HCM not manifesting in offsprings
B. HCM disappearing with advancing age
C. HCM progressing to DCM with disappearance of preexisting outflow pressure gradient
D. HCM with AR

Hypertrophic cardiomyopathy

[MCQ] Hypertrophic cardiomyopathy (HCM)

I. Start the exam by click the “Start” button

Hypertrophic cardiomyopathy (HCM)

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