Echocardiographic Evaluation of Cardiomyopathies

Which one of the following primary cardiomyopathies is most common?

Dilated

Hypertrophic

Infiltrative

Restrictive

Dilated cardiomyopathy can typically be distinguished from other primary cardiomyopathies by the presence of which one of the following?

Decreased ejection fraction

Impaired left ventricular relaxation

Decreased end-diastolic volume

Mitral regurgitation

Which one of the following locations is the most common site for the development of thrombi in patients with dilated cardiomyopathy?

Main pulmonary artery

Left atrium

Left ventricular apex

Right atrial appendage

All of the following factors are associated with decreased 5-year survival following the initial diagnosis of dilated cardiomyopathy except:

Biventricular dysfunction

Childhood onset

Delayed initiation of therapy

Increased extent of cardiac remodeling

What percentage of patients with dilated cardiomyopathy will die within 2 years?

20% to 25%

45% to 50%

70% to 75%

90% to 95%

What percentage of patients with dilated cardiomyopathy improve spontaneously?

20% to 25%

45% to 50%

70% to 75%

90% to 95%

Idiopathic dilated cardiomyopathy can most commonly be distinguished from ischemic cardiomyopathy by which one of the following characteristics?

Absence of coronary artery disease

Angina

Improvement in regional ventricular wall motion following dobutamine

Symmetric and globally reduced ventricular wall motion

Which one of the following echocardiographic findings pertaining to left ventricular dilatation in patients with dilated cardiomyopathy is correct?

Basal diameter exceeds midventricular diameter.

Decreased end-systolic diameter.

Diminished relative wall thickness.

Left ventricular dilatation is usually less than right ventricular dilatation.

Which of the following echocardiographic findings is most consistent with a diagnosis of right ventricular dilatation?

Apex-forming right ventricle

Hepatic vein Doppler flow velocity reversal during systole

Right ventricular free wall akinesis

Tricuspid regurgitation

Which of one the following echocardiographic findings is fundamental to the diagnosis of dilated cardiomyopathy?

Left ventricular end-systolic diameter less than 4 cm2

Left ventricular ejection fraction less than 45%

Right ventricular dilatation

Severe mitral regurgitation

Which one of the following echocardiographic findings is consistent with the greatest risk for systemic embolization of thrombus?

Dilated left atrium

Left atrial appendage laminated thrombus

Protuberant left ventricular apical thrombus

Spontaneous contrast in the left ventricle

Diagnostic echocardiographic criteria for identifying a left ventricular thrombus in a patient with dilated cardiomyopathy include all of the following, except:

Demarcation by a clear thrombus-blood interface

Normal transmitral Doppler flow velocity profile

Usual location adjacent to but distinct from abnormally contracting myocardium

Visualization in at least two planes

In patients with dilated cardiomyopathy, impaired left ventricular relaxation is indicated by which one of the following characteristics of the transmitral Doppler flow velocity profile?

Decreased A-wave velocity

Decreased isovolumic relaxation time

Increased deceleration time

Increased E-wave velocity

In patients with dilated cardiomyopathy, impaired left ventricular relaxation is indicated by which one of the following characteristics of the pulmonary venous Doppler flow velocity profile?

Atrial reversal component duration exceeding the transmitral A-wave duration

Atrial reversal velocity greater than 35 cm/s

Peak diastolic velocity less than peak systolic velocity

Systolic flow reversal

In patients with dilated cardiomyopathy, mitral regurgitation is most commonly due to which one of the following mechanisms?

Apical displacement of the coaptation point

Mitral annular calcification

Posterior leaflet prolapse

Systolic anterior motion

All of the following echocardiographic findings have been correlated with a worse outcome in patients with dilated cardiomyopathy, except:

Decreased left ventricular end-systolic volumes following dobutamine

Increased tricuspid regurgitation peak Doppler flow velocity

Restrictive transmitral Doppler flow velocity profile

Severe mitral regurgitation

In patients with dilated cardiomyopathy, decreased right ventricular compliance is indicated by which one of the following echocardiographic findings?

Increased transtricuspid E-wave deceleration time

Inferior vena cava diameter less than 1.5 cm2

Decreased transtricuspid peak E-wave velocity

Predominant hepatic vein V- and A-wave reversals during spontaneous inspiration

In patients with dilated cardiomyopathy, which one of the following most likely accounts for the relative increased incidence of thrombi found in the left ventricle compared to the left atrium?

Atrial fibrillation

Concurrent left ventricular apical myocardial infarction

Decreased left atrial compliance

Protective effect of mitral regurgitation

All of the following echocardiographic findings are commonly associated with dilated cardiomyopathy, except:

Dilated left atrium

Impaired left ventricular relaxation

Focal regional wall motion abnormalities

Mitral regurgitation

Etiologies for the development of dilated cardiomyopathy include all of the following, except:

Familial linkage

Autoimmunity

Systemic hypertension

Viral myocarditis

Primary cardiomyopathies:

Can be associated with coronary artery narrowing

Are associated with systemic disease

Are commonly found in the general population

Always involve all four chambers

Are always associated with diastolic dysfunction

True statements regarding primary cardiomyopathies include all of the following, except:

They overlap with each other with respect to clinical symptoms.

All may have diastolic dysfunction.

All may have systolic dysfunction.

They are associated with a systemic disease.

The incidence in the general population is less than 1%.

All patients with hypertrophic cardiomyopathy have a globally hypertrophied ventricle.

True

False

Approximately 25% of patients with hypertrophic cardiomyopathy have left ventricular outflow tract obstruction.

True

False

All of the following contribute to systolic anterior motion and left ventricular outflow tract obstruction, except:

Posterior mitral annular calcification

Septal hypertrophy

Elongated mitral leaflets

Direction of ventricular inflow

Posterior shift of papillary muscle

The incidence of HCM/HOCM in the general population is:

Less than 1%

Less than restrictive/infiltrative cardiomyopathy

Greater than 10%

Between 1% and 10%

Greater than for dilated cardiomyopathy

Genetic testing for patients with HCM/HOCM:

Has found abnormalities on only one gene

Is performed in all patients

Has found mutations on at least nine genes

Has not revealed any genetic abnormalities

Is routinely performed in all family members of patients with HCM/HOCM

All patients with ventricular hypertrophy:

Have a primary cardiomyopathy

Have an asymmetric pattern of hypertrophy

Have aortic stenosis and/or systemic hypertension

Have involvement of the interatrial septum

Are more likely to have echocardiographic evidence of diastolic dysfunction

All of the following are true except:

Hypertrophic cardiomyopathy may be symptomatic or asymptomatic.

Diastolic dysfunction is found in the majority of patients with ventricular hypertrophy.

Systolic outflow obstruction can be dynamic and episodic.

Restrictive filling is found only in restrictive cardiomyopathy.

Varying degrees of diastolic dysfunction are found in patients with hypertrophy.

Hypertrophic cardiomyopathy:

Incidence is greatest in the elderly patient

Incidence is greatest in patients under 10 years old

Can occur only after the second decade

Is more commonly discovered between the 2nd and 5th decades

Does not predict risk of complications

Risk factors for sudden death in hypertrophic cardiomyopathy include all of the following, except:

Personal or family history of syncope

Presence of left ventricular outflow tract obstruction

Young patients

Severe left ventricular hypertrophy (>20-mm thickness)

A reduction in blood pressure with exercise

Relationships between the mitral coaptation point and the left ventricular outflow tract that predispose to outflow tract obstruction include:

Distance between the mitral coaptation point and the ventricular septum 25 mm or less

Mitral inflow/aortic outflow angle of less than 30°

Relative lengths of the anterior and posterior leaflets of greater than 2.0:1.0

Distance between the mitral annulus and septum less than 25 mm

Posterior displacement of the coaptation point

For hypertrophic cardiomyopathies, coronary ischemia may be due to all of the following, except:

Hypertrophy of the coronary arteries

Elevated intraventricular pressures

Coronary artery aneurysm and thrombosis

Coronary atheromatous disease

Reduced coronary vasodilatory reserve

Which of the following is least likely to be performed as a primary treatment in patients with hypertrophic cardiomyopathy?

Mitral valve replacement with a bioprosthetic valve

Placement of an internal cardiac defibrillator

Mitral valve repair

Surgical resection of the hypertrophic ventricular septum

Administration of antibiotic prophylaxis

True statements regarding hypereosinophilic syndrome include all of the following, except:

It is also known as Loffler endocarditis.

It is continuous with endomyocardial fibrosis (ie, same disease).

It is known to cause endocardial thickening of the mitral and tricuspid annuli.

It is associated with significant systolic dysfunction.

Endocardial thickening is associated with obliteration of the ventricular apex.

Differential diagnosis of restrictive filling defect include all of the following, except:

Myocardial ischemia/infarct

Cardiac amyloidosis

Mitral regurgitation

Hypereosinophilic syndrome

End-stage aortic stenosis

Histologic examination of hypertrophic cardiomyopathy may include all of the following, except:

Myocardial disarray

Myocardial fibrosis

Myocardial hypertrophy

Amyloid infiltration

Asymmetric distribution of hypertrophy

Doppler data suggestive of restrictive filling pattern include all except:

Transmitral deceleration time less than 150 milliseconds

Isovolumic relaxation time less than 80 milliseconds

Transmitral early to late filling fraction ratio (E/A) less than 0.70

Pulmonary venous systolic to diastolic inflow ratio (E/A) of less than 0.5

Pulmonary venous atrial reversal (Arev) velocity greater than 35 cm/s

Which of the following statements is true regarding patients with hypertrophic cardiomyopathy?

Hypertrophy is frequently found in the right ventricle.

Hypertrophy is not found in the inferolateral wall.

Hypertrophy is typically asymmetric with greater involvement of the septum.

Hypertrophy typically involves the interatrial septum.

Which of the following statements is true regarding patients with hypertrophic cardiomyopathy?

Coronary arteries may exhibit abnormal concentric thickening.

Always coexists with pulmonary hypertension.

It is inherited as an autosomal recessive trait with variable expression.

Diastolic dysfunction occurs in 25% of patients.

In patients with hypertrophic cardiomyopathy, ventricular hypertrophy can involve:

Basilar septum

Inferolateral wall

Mid-cavity

Apex

All of the above

Which of the following echocardiogarphic findings is most likely associated with hypertrophic cardiomyopathy?

Increased intracavitary dimensions

Decreased systolic function

Symmetric ventricular hypertrophy

Systolic anterior motion of the mitral valve

Which of the following statements is true regarding echocardiographic evidence of left ventricular outflow tract obstruction?

M-mode demonstrates late closure of the aortic valve.

M-mode demonstrates premature opening of the aortic valve.

M-mode demonstrates late opening of the aortic valve.

M-mode demonstrates premature closure of the aortic valve.

Which of the following statement is true regarding patients with hypertrophic cardiomyopathy?

Ventricular dysrhythmias are the main cause of death.

Left ventricular outflow obstruction has been directly related the sudden death.

Left ventricular outflow obstruction is directly related to arrhythmias.

The annual mortality rate ranges from 0.5% to 1%.

Which of the following increases the risk of sudden death in patients with hypertrophic cardiomyopathy?

LVOT gradient of 35 mm Hg

Reduction in blood pressure during exercise

LV hypertrophy of 20 mm

Old age

With regard to systolic anterior motion of the mitral valve and outflow tract obstruction:

It is found in approximately 70% of patients with hypertrophic cardiomyopathy.

It has different mechanisms across different populations.

It is related, in part, to abnormalities of the mitral apparatus.

The Bernoulli effect is responsible for outflow tract obstruction.

M-mode echocardiography is useful for the evaluation of hypertrophic cardiomyopathy by detecting or measuring:

Ventricular diastolic function

Ventricular wall thickness

Mitral insufficiency when combined with color Doppler

Aortic insufficiency when combined with color Doppler evaluation of the left ventricular outflow tract

True statements regarding diastolic dysfunction include:

It occurs in all patients with hypertrophic cardiomyopathy.

Doppler assessment is often affected by changes in loading conditions.

It is always associated with systolic dysfunction.

It occurs in 25% of patients with restrictive cardiomyopathy.

Which of the following is associated with a restrictive cardiomyopathy?

Myocarditis

Friedreich ataxia

Lead poisoning

Cardiac hemochromatosis

Typical echocardiographic features in patients with restrictive cardiomyopathy include:

Left ventricular free-wall thickening

Right ventricular free-wall thickening

Decreased LV systolic function

Normal right and left atrial size

The typical mitral inflow velocity in patients with restrictive cardiomyopathy includes:

Short IVRT, tall E wave, short DT

Short IVRT, short E wave, short DT

Short IVRT, tall E wave, long DT

Short IVRT, short E wave, long DT

The prognosis for patients with cardiac amyloidosis is:

Poor for patients with a pulmonary venous systolic/ diastolic inflow ratio less than 0.5

Poor for patients with LV wall thickness in diastole greater than 15 mm

Poor for patients with a transmitral deceleration time less than 140 milliseconds

All of the above

When differentiating cardiac amyloidosis from other causes of restrictive patterns:

Speckling or a “starry-skied” appearance of the myocardium is found only in patients with amyloidosis.

Left ventricular systolic dysfunction is more likely to be present in amyloidosis.

Involvement of the interatrial septum is strongly suggestive of amyloid infiltration.

Dilation of the atria is diagnostic of amyloidosis.

In patients with amyloid infiltration of the heart:

The incidence of left ventricular systolic dysfunction is not related to wall thickness.

Left ventricular systolic dysfunction is present in 70% of cases.

Left ventricular dilation is present in less than 10% of cases with systolic dysfunction.

Biventricular dilation is an end-stage event.

Cardiac involvement in hemochromatosis:

Commonly presents with restrictive pathophysiology

Is commonly seen without other organ system involvement

Uncommonly contributes to mortality

Is associated with systolic dysfunction and chamber enlargement

Which of the following statements is correct regarding constrictive pericarditis and restrictive cardiomyopathy?

Both have a similar clinical presentation.

Both have decreased LV systolic function.

Both have the same pattern of impaired diastolic filling.

Both are associated with pulmonary hypertension.

Echocardiographic data that differentiate constrictive pericarditis from restrictive cardiomyopathy include:

Respiratory variation of transmitral and pulmonary venous flows

Presence of a thickened and/or calcified pericardium

Normal-appearing interatrial septum

All of the above

Intracardiac thrombi may occur:

With all three categories of cardiomyopathy

With abnormalities of the endocardium due to infiltrative diseases

In the dilated and dysfunctional atrium

In areas of low flow velocity (<25 cm/s)

All of the above