Cardiomyopathies Flashcards
Functional classifications of cardiomyopathy include all the following EXCEPT:
A. Dilated
B. Hypertrophic
C. Non-dilated
D. Restrictive
C. A variety of schemes have been proposed for classifying the cardiomyopathies. Three basic categories of functional impairment have been described: (1) dilated (formerly called congestive), characterized by ventricular dilatation, contractile dysfunction and often symptoms of congestive heart failure; (2) hypertrophic, recognized by inappropriate left ventricular hypertrophy, often with asymmetrical involvement of the septum, with preserved or enhanced global systolic ventricular function; and (3) restrictive, marked by endocardial scarring of the ventricle, with impairment of diastolic filling. The distinctions between these categories are not absolute.
Two less common forms of cardiomyopathy are recognized: arrhythmogenic right ventricular cardiomyopathy (ARVC) and unclassified which includes systolic dysfunction with minimal dilatation and isolated ventricular noncompaction.
A more appropriate name for idiopathic hypertrophic cardiomyopathy (IHSS) is:
A. Aortic tunnel disease (ATD)
B. Discrete subaortic stenosis (DSS)
C. Hypertrophic cardiomyopathy
D. Subaortic hourglass deformity (SHD)
C. The characteristic finding in hypertrophic cardiomyopathy is inappropriate myocardial hypertrophy most often involving the interventricular septum (asymmetrical septal hypertrophy, ASH) which may result in a left ventricular outflow tract obstruction. (hypertrophic obstructive cardiomyopathy, HOCM).
The types include obstructive, provokable obstructive, non-obstructive, mid-ventricular obstruction and asymmetric apical hypertrophy (AAH).
Although the interventricular septum is the most common location for hypertrophy, concentric hypertrophy, apical and/or free wall hypertrophy can be present.
M-mode findings associated with hypertrophic cardiomyopathy include all of the following EXCEPT:
A. Asymmetric septal hypertrophy (ASH)
B. Mid-systolic notching of the aortic valve
C. Mid-systolic notching of the pulmonary valve
D. Systolic anterior motion of the mitral valve (SAM)
C. Although two-dimensional echocardiography is the method of choice for evaluating hypertrophic cardiomyopathy, M-mode echocardiography may be useful in identifying the presence of mitral valve systolic anterior motion (SAM) and mid-systolic notching of the aortic valve.
Mid-systolic notching (flying W) of the pulmonary valve is associated with pulmonary hypertension.
The mitral valve finding most strongly associated with hypertrophic obstructive cardiomyopathy is mitral valve:
A. Aneurysm
B. Systolic anterior motion
C. Fenestration
D. Flail leaflet
B. Systolic anterior motion (SAM) is seen as an abrupt anterior motion of the mitral valve which starts a short time after the closure of the mitral valve and lasts throughout systole ending just before the mitral valve opens. The duration of systolic anterior motion does vary.
Systolic anterior motion of the mitral valve is thought to be the reason for left ventricular outflow tract obstruction in hypertrophic obstructive cardiomyopathy.
A hallmark M-mode aortic valve finding in patients with hypertrophic obstructive cardiomyopathy is aortic valve:
A. Diastolic flutter
B. Fine systolic flutter
C. Mid-systolic notching
D. Vegetation
C. Mid-systolic notching of the aortic valve is best seen on M-mode echocardiography of the aortic valve. It is often associated with coarse systolic leaflet flutter. The aortic valve opens in early systole closes partially in mid-systole and then moves back to a fully open position.
This appears to be a specific and sensitive echocardiographic finding in patients with hypertrophic obstructive cardiomyopathy.
Fine systolic flutter of the aortic valve is a normal finding.
Diastolic flutter of the aortic valve suggests significant aortic regurgitation.
Common two-dimensional echocardiographic findings in hypertrophic obstructive cardiomyopathy include all of the following EXCEPT:
A. Asymmetric interventricular septal hypertrophy
B. Systolic anterior motion of the mitral valve
C. Left atrial enlargement
D. Left ventricular enlargement
D. In hypertrophic cardiomyopathy the left ventricular cavity is usually small and there may even be left ventricular cavity obliteration during ventricular systole. Other two-dimensional findings include hypokinesis of the interventricular septum, speckled appearance of the interventricular septum, thickening of the mitral valve, mitral valve prolapse, mitral annular calcification, endocardial plaqueing of the interventricular septum and right ventricular hypertrophy.
A speckled or ground-glass appearance of the interventricular septum seen on two-dimensional echocardiography is found is:
A. Constrictive pericarditis
B. Mitral stenosis
C. Dilated cardiomyopathy
D. Hypertrophic cardiomyopathy
D. Because of the myocardial fiber disarray hypertrophic cardiomyopathy presents a speckling or ground-glass appearance of the interventricular septum. This speckling may also be seen in cardiac amyloidosis, systemic hypertension, chronic renal failure and hypothyroidism.
Pulsed-wave Doppler and color flow Doppler are useful in hypertrophic cardiomyopathy in all of the following ways EXCEPT:
A. Aid in guiding the continuous-wave Doppler beam
B. Determine the presence and severity of mitral regurgitation
C. Help distinguish left ventricular outflow tract flow from mitral regurgitation
D. Quantitate the severity of the left ventricular outflow tract obstruction
D. Pulsed-wave Doppler and color flow Doppler aid in locating the site of obstruction. Continuous-wave Doppler allows for the resolution of high velocity flow. A gradient of > 50 mm Hg is usually considered severe in hypertrophic cardiomyopathy.
Nearly 90% of patients with systolic anterior motion have mitral regurgitation. Differentiating the outflow tract obstruction flow from mitral regurgitation may be difficult. Mitral regurgitation is usually a higher velocity than the left ventricular outflow tract obstruction flow and mitral regurgitation extends to mitral valve opening while the outflow tract obstruction flow does not because there is no flow in the outflow tract during isovolumic relaxation period.
A systolic high-velocity, late-peaking, dagger-shaped continuous-wave Doppler signal is obtained. The most likely diagnosis is:
A. Hypertrophic obstructive cardiomyopathy
B. Mitral regurgitation
C. Tricuspid regurgitation
D. Valvular aortic stenosis
A. The late peaking dagger-shaped systolic flow velocity pattern is a classic finding for a dynamic obstruction such as hypertrophic obstructive cardiomyopathy.
All of the following maneuvers may induce or enhance the obstruction in hypertrophic cardiomyopathy EXCEPT:
A. Supine to standing
B. Valsalva maneuver
C. Inhalation of amyl nitrate
D. Leg raising
D. A maneuver which reduces venous return may be used to establish obstruction in hypertrophic cardiomyopathy. An enhancement of the obstruction may also be seen after a meal, post-premature ventricular contraction (e.g., postextrasystolic potentiation) and with the ingestion of alcohol.
The pulsed-wave Doppler mitral flow pattern most often associated with hypertrophic cardiomyopathy is grade:
A. I
B. II
C. III
D. IV
A. Although any diastolic grade can be present in hypertrophic cardiomyopathy the impaired relaxation flow pattern (Grade I) is the predominant one. Grade I presents as a reduced mitral valve E/A ratio (< 0.75), increased deceleration time (> 220 msec), increased isovolumic relaxation time (> 100 msec) and a reduced mitral annular tissue Doppler E’ wave (< 8 cm/sec).
The E/E’ ratio may be a useful indicator of filling pressures in patients with hypertrophic cardiomyopathy.
When examining young patients a normal pulsed-wave Doppler E/A ratio would be > 1.5. A Grade I flow pattern (E/A ratio < 75) is an important finding.
All of the following are considered possible pharmacologic treatment for hypertrophic obstructive cardiomyopathy EXCEPT:
A. Beta-blockers
B. Calcium-channel blocker (e.g., Verapamil)
C. Disopyramide
D. Digitalis
D. Beta-blockers are usually the first choice and are usually effective in 60 to 80% of patients. Disopyramide has a negative inotropic effect which may reduce the severity of the outflow tract obstruction. Verapamil may be used with caution.
All of the following may be used to treat hypertrophic obstructive cardiomyopathy EXCEPT:
A. Septal myectomy
B. Dual chamber pacemaker
C. Alcohol-induced septal ablation
D. Aspirin
D. Septal myectomy is the resection of the basal septum. Pacing results in paradoxical interventricular septal motion which may reduce the severity of the obstruction. Alcohol-induced septal ablation is a newer method, produces a controlled myocardial infarction with the thinning and remodeling of the interventricular septum resulting in a reduction of the outflow tract obstruction.
The cardiomyopathy described as presenting with four-chamber enlargement with poor global ventricular systolic function is:
A. Dilated
B. Hypertrophic
C. Restrictive
D. Uhl’s
A. Etiologies of dilated cardiomyopathy include idiopathic, familial, alcohol, drugs (e.g., cocaine) chemotherapy (e.g., adriamyacin), viral, post-partum, severe coronary artery disease (e.g., ischemic cardiomyopathy).
Characteristic findings in patients with dilated cardiomyopathy include all of the following EXCEPT:
A. Asymmetric septal hypertrophy
B. Dilated, poorly contracting left ventricle
C. Low cardiac output
D. Increased intracardiac pressures
A. The classic description of dilated cardiomyopathy is four-chamber enlargement with poor global ventricular systolic function. All systolic indices including fractional shortening, ejection fraction or fractional area change are reduced. Wall thickness remains normal or “thin” but left ventricular mass may be increased.
Echocardiographic findings in dilated cardiomyopathy include all of the following EXCEPT:
A. Apical mural thrombus
B. Dilated ventricular cavities
C. Enlarged atrial cavities
D. Preserved ejection fraction
D. In dilated cardiomyopathy the left ventricle is dilated with poor global ventricular systolic function with an ejection fraction of < 40%.
Other findings in dilated cardiomyopathy include dilatation of the mitral annulus, papillary muscle dysfunction with tenting of the mitral valve leaflets, low cardiac output, valvular regurgitation and diastolic dysfunction.
A common mitral valve finding in dilated cardiomyopathy in two-dimensional echocardiography is:
A. Decreased E-septal separation
B. Reversed diastolic doming
C. Incomplete closure of the mitral valve
D. Premature closure of the mitral valve
C. A common finding with dilated cardiomyopathy is incomplete closure of the mitral valve due to the change in the shape of the left ventricle. This incomplete closure of the mitral valve may also be referred to as tenting of the mitral valve and may be best seen in the apical four-chamber view. Tenting of the mitral valve is a possible explanation for the common finding of mitral regurgitation in dilated cardiomyopathy.
Tenting of the tricuspid valve may also occur, is best evaluated in the apical four-chamber view and may be the explanation for tricuspid regurgitation in dilated cardiomyopathy.
A common late complication associated with dilated cardiomyopathy is:
A. Infective endocarditis
B. Mitral regurgitation
C. Systemic emboli
D. Ventricular gallop
C. Common late complications include (1) systemic emboli resulting from intracardiac thrombi dislodged from the left ventricle and/or left atrium and (2) pulmonary emboli that originate in the peripheral venous system, right ventricle and/or right atrium.
The most common regurgitation found in patients with dilated cardiomyopathy is:
A. Aortic regurgitation
B. Mitral regurgitation
C. Pulmonary regurgitation
D. Tricuspid regurgitation
B. Mitral regurgitation is present in nearly 100% of patients with dilated cardiomyopathy. Tricuspid regurgitation is present in nearly 90%, pulmonary regurgitation in 50% and aortic regurgitation in 20%.
Atrioventricular valve regurgitation results from enlargement of the circumference of the mitral or tricuspid valve annulus and ventricular dilatation with resultant distortion of the geometry of the subvalvular apparatus.
Early in the disease stage the usual pulsed-wave Doppler flow of the mitral valve in patients with dilated cardiomyopathy demonstrates a Grade:
A. I
В. ІI
С. III
D. IV
A. Mitral inflow is invariably abnormal in patients with dilated cardiomyopathy. Early in the disease state the usually mitral flow pattern is impaired relaxation (Grade I) (e.g., reduced E/A ratio). As the left ventricular diastolic pressures increase the Grade I pattern may progress toward the restrictive pattern (Grade III or IV) (increased E/A ratio). During this progression, one may recognize a pattern of pseudonormalization (Grade II) (normal E/A ratio).
Possible causes of restrictive cardiomyopathy include all of the following EXCEPT:
A. Alcohol
B. Amyloidosis
C. Hemochromatosis
D. Sarcoidosis
A. The two categories of restrictive cardiomyopathy are endomyocardial fibrosis and infiltrative myocardial disease which includes amyloidosis, sarcoidosis, hemochromatosis, Pompe’s disease and Fabry’s disease.
Restrictive cardiomyopathy is the least common type of cardiomyopathy.
Amyloidosis is the most common restrictive cardiomyopathy in the United States. Endomyocardial fibrosis is the most common outside the United States and is more common in children.
Excessive alcohol consumption may result in dilated cardiomyopathy.
The echocardiographic features of amyloidosis include all of the following EXCEPT:
A. Dilatation of the ascending aorta
B. Increased venricular wall thickness
C. Multivalvular regurgitation
D. Pericardial effusion
A. Amyloidosis is a systemic disease resulting from deposition of amyloid in the myocardium resulting in a restrictive cardiomyopathy. Other echocardiographic/Doppler features include granular ground-glass appearance of the myocardium, thickening of the cardiac valves, normal ventricular size, dilated atria, pleural effusion and diastolic dysfunction.
The cardiomyopathy with which cardiac hemochromatosis is most commonly associated with is:
A. Dilated
B. Hypertrophic
C. Idiopathic
D. Arrhythmogenic right ventricular cardiomyopathy
A. Idiopathic hemochromatosis is characterized by large deposits of iron in various organs including the heart. The echocardiographic/Doppler findings include a dilated left ventricle with reduced global systolic function and abnormal diastolic function.
Possible echocardiographic findings for sarcoid heart disease include:
A. Asymmetric septal hypertrophy
B. Concentric left ventricular hypertrophy
C. Dilatation of the ascending aorta
D. Infero-basal aneurysm
D. Sarcoidosis is a granulomatosis disease of unknown etiology.
Echocardiographic/Doppler features include dilated cardiomyopathy, regional wall motion abnormalities, wall thinning of the basal inferior and lateral walls with possible aneurysm formation (e.g., infero-basal aneurysm) and abnormal diastolic function.
The primary cause of endomyocardial fibrosis is:
A. Intravenous drug abuse
B. Chemotherapy
C. Hypereosinophilia
D. Alcohol
C. There are two basic types of endomyocarial fibrosis. One type is due to hypereosinophilia and is called hypereosionophilic syndrome, Loffler’s endocarditis or Loffler’s endocarditis parietalis fibrolastic syndrome. This type usually affects males in their 40’s with increased eosinophils of more than 1500/mm’.
The second type of endomyocardial fibrosis is termed obliterative endomyocardial fibrosis and occurs in most commonly in East Africa.
Echocardiographic/Doppler findings for endomyocardial fibrosis include endocardial thickening of the inflow tracts, thrombotic-fibrotic obliteration of the apices, atrioventricular regurgitation and abnormal diastolic function.
The progressive replacement of right ventricular myocardium with fatty and fibrous tissue is called:
А. НСМ
B. ARVC
C. IHSS
D. DSS
B. Arrhythmogenic right ventricular cardiomyopathy (ARVC), also called arrhythmogenic right ventricular dysplasia (ARVD), is the most common cause of death in young patients in certain areas of Europe.
It is familial. The echocardiographic/Doppler features include right ventricular dilatation with reduced global right ventricular systolic function, right ventricular wall motion abnormalities especially at the right ventricular apex and anterior wall) and low velocity (< 2 m/s due to poor global right ventricular systolic function) tricuspid regurgitation.
Persistent intramyocardial sinusoids located in the left ventricle are found in:
A. Dilated cardiomyopathy
B. Hypertrophic cardiomyopathy
C. Restrictive cardiomyopathy
D. Noncompaction cardiomyopathy
D. Isolated ventricular noncompaction cardiomyopathy is characterized by a dilated left ventricle with reduced global systolic function with thick epicardial and endocardial layers and heavy trabeculations with deep intrabecular recesses.
The cardiac involvement associated with acquired immunodeficiency syndrome (AIDS) is:
A. Dilated cardiomyopathy
B. Hypertrophic cardiomyopathy
C. Restrictive cardiomyopathy
D. Arrhythmogenic right ventricular cardiomyopathy
A. Other echocardiographic/Doppler findings in AIDS patients include pericardial effusion, cardiac tamponade, infective endocarditis, metastatic cardiac neoplasm and pulmonary hypertension.
