Cardiomyopathies

Question 1

Imaging findings Fabry’s disease

Answer 1

LVH

Focal mid myocardial LGE - basal inferolateral location

Question 2

Imaging findings Churg-Strauss

Answer 2

Delayed myocardial enhancement - variable pattern (mid myocardial, epicardial, subendocardial) +/- edema

Reduced LVEF

Question 3

Enhancement pattern DCM

Answer 3

Mid interventricular septum

Question 4

What is athletes heart?

Answer 4

• biventricular hypertrophy and dilation
• preservation of LV:RV size ratio and LV volume:LV mass ratio
• biatrial enlargement
• aortic root dilation

*adaptations in heart structure and function in an athletes heart

Question 5

Causes of DCM

Answer 5

• Primary/familial
• Secondary (infection/viral, ischemia, infiltrative disease, peripartum, HIV, alcohol)

*50% are idiopathic

Question 6

Inheritance pattern HCM

Answer 6

AD, variable penetrance (50%)

Remainder are sporadic

Question 7

Key ECHO findings HCM

Answer 7

Anterior motion of the mitral valve during systole (SAM), asymmetric hypertrophy

*can have isolated apical HCM (better prognosis, rarely associated with sudden cardiac death)

Question 8

Causes of restrictive cardiomyopathy

Answer 8

Primary/idiopathic

Secondary - amyloid, hemochromatosis, sarcoid, scleroderma, Loeffler (hypereosinophilic )

Question 9

Imaging findings in restrictive cardiomyopathy

Answer 9

Right heart failure, reduced diastolic filling (systolic function preserved), normal size ventricles, dilated atria

Question 10

Imaging findings cardiac sarcoidosis

Answer 10

Midmyocardial and epicardial enhancement in non-vascular distribution - often involves septum and LV wall (RV wall and papillary muscles usually spared)

DDx: amyloid (usu more diffuse, involves papillary muscles), myocarditis