2B Class Reading Knowledge Assessment - Restrictive, Unclassified CM

1 wrong (will fix

Amyloidosis is an infiltrative cardiomyopathy where the myocardium is infiltrated with an excess of __ .

protein

Endomyocardial fibrosis is an infiltration of __ that lines the inner lining of the myocardium.

fibrotic tissue

__ causes excessive glycogen storage in the tissues.

Pompes

The sonographer should rule out areas of necrosis in the patient with endomyocardial fibrosis.

True

With restrictive cardiomyopathy, systolic function is normal and heart failure symptoms are due to diastolic dysfunction, also known as:   

HFpEF

In chronic restrictive cardiomyopathies, systolic dysfunction may occur. 

True

Stiffening of the LV transmits the diastolic pressure to the LA and pulmonary veins resulting in extreme atrial dilation with relatively preserved LVID. 

True

Scanning in both harmonic and fundamental frequencies may be useful to distinguish 'ground glass' texture of myocardium in restrictive cardiomyopathies. 

True

Apical sparing of global longitudinal strain has been reported as a specific marker of cardiac amyloid in comparison to other hypertrophic pathologies. 

True

In advanced restrictive cardiomyopathy, this would be a common E/A pattern. 

False

In early stages of restrictive cardiomyopathy, this would be a common E/A ratio. 

False

Muscular dystrophies and Friedrich ataxia may have cardiac involvement and would be important clinical history information. 

True

Uhl Disease in an acquired abnormality of the LV seen in advanced age.

False

Uhl Disease may demonstrate a 'banana' shaped LV such as this image below:

True

__ may present with concentric and asymmetric hypertrophy mimicking hypertrophic cardiomyopathy and regional wall motion abnormalities that may mimc CAD. 

Fabry disease

Which of the following is a common restrictive/infiltrative cardiomyopathy finding?

stiff, rigid ventricular walls

biatrial enlargement

resembles constrictive pericarditis

All of the above

What disease process typically occurs early in life causing cardiac enlargement and heavily thickened due to excessive glycogen storage in the tissues?

Pompe disease

What technique is utilized to provoke LV outflow tract obstruction?

coughing

Valsalva maneuver

exercise

All of these

A 19-year-old presents with chest pain and shortness of breath. A small, hyperdynamic LV with LV brightening is demonstrated. IVS/PW thickness ratio = 1.7. SAM/LVOT peak gradient is 64 mmHg. There is moderate MR and LA volume index is 46 mL/m2.

What is the most likely diagnosis?

asymetric septal hypertrophy, systolic anterior motion of the mitral valve, and LVOTO