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Restrictive cardiomyopathy
See all quizzes of the Restrictive cardiomyopathy at here:
1. The hallmark of the late secondary restrictive cardiomyopathies is abnormal ?
A. Systolic dysfunction
B. Diastolic dysfunction
C. Systolic + diastolic dysfunction
D. Any of the above
2. Which of the following is a feature of restrictive cardiomyopathy ?
A. Both atria are enlarged
B. End-diastolic pressures are elevated in both ventricles
C. Preserved cardiac output
D. All of the above
3. Which of the following is the more common cause of secondary restrictive cardiomyopathy ?
A. Amyloidosis
B. Sarcoidosis
C. Scleroderma
D. Hemochromatosis
4. Familial amyloidosis results from an autosomal dominant mutation in ?
Harrison’s 18th Ed. 1964
A. Calcitonin
B. Gelsolin
C. Transthyretin
D. Amyloid protein
5. Amyloidosis is best related to which of the following ?
A. Glycosphingolipid
B. Congo red stain
C. Mucopolysaccharidoses
D. Hypereosinophilic syndrome
6. Amyloid fibrils infiltrate the myocardium, especially around ?
A. Cardiomyocyte
B. Coronary vessels
C. Lymphatics
D. Entry of SVC, IVC
7. Typical feature of amyloidosis is ?
A. Conduction block
B. Autonomic neuropathy
C. Renal involvement
D. All of the above
8. 2D echocardiogram that shows a thickened myocardial wall with a distinctive “speckled” appearance is suggestive of ?
A. Endomyocardial fibrosis
B. Eosinophilic endomyocardial disease
C. Primary cardiac amyloidosis
D. All of the above
9. Which of the following go in favour of amyloidosis ?
A. Low voltage ECG
B. Refractile brightness in septum on echocardiography
C. Both atria are dilated
D. All of the above
10. Fabry’s disease results from a deficiency of ?
A. Alpha-galactosidase A
B. Beta-galactosidase
C. Sphingomyelinase
D. Neuraminidase
11. Which of the following about Fabry’s disease is false ?
A. Autosomal dominant disorder
B. Disorder of glycosphingolipid metabolism
C. Leg lymphedema without hypoproteinemia
D. Enzyme therapy useful
12. Which of the following about angiokeratomas in Fabry’s disease is false ?
A. Do not blanch with pressure
B. Mostly between umbilicus & knees “bathing suit area”
C. Punctate, dark red to blue-black
D. Asymmetric
13. Cardiac Danon’s Disease is due to mutations in ?
A. LAMP1
B. LAMP2
C. LAMP3
D. LAMP4
14. ECG of which of the following inherited metabolic cardiomyopathies with LVH shows ventricular preexcitation ?
A. Fabry Disease
B. Friedreich’s Ataxia
C. Cardiac Danon Disease
D. Glycogen Storage Cardiomyopathy
15. Differentiation of constrictive pericarditis from restrictive cardiomyopathies is done by which of the following ?
A. Apex impulse
B. Mitral regurgitation
C. Pericardial calcification
D. All of the above
16. Endomyocardial fibrosis most commonly affects which of the following age groups ?
A. Children and young adults
B. Middle aged
C. Elderly
D. Any of the above
17. Fibrous endocardial lesions in endomyocardial fibrosis involve which of the following areas of heart ?
A. Inflow portion of right or left atria (or both)
B. Outflow portion of right or left atria (or both)
C. Inflow portion of right or left ventricle (or both)
D. Outflow portion of right or left ventricle (or both)
18. Endocardial fibrosis results from which of the following ?
A. Carcinoid syndrome
B. Fenfluramine
C. Phentermine
D. All of the above
