1. Cardiac p34 (Genetic)

Question 1

Arrhythmogenic Right Ventricular Cardiomyopathy - definition

Answer 1

Fibrofatty degeneration of RV leading to arrhythmias and sudden death

Question 2

ARVC - anatomy (3)

Answer 2

Dilated RV with reduced function
Fibrofatty replacement of myocardium
Normal LV

Question 3

ARVC - imaging

Answer 3

Fat-sat MRI to demonstrate far in RV wall

Question 4

Hypertrophic cardiomyopathy - definition/trivia (2)

Answer 4

Abnormal hypertrophy of myocardium that compromises diastole.
Cause of sudden death.

Question 5

Hypertrophic cardiomyopathy - types (2)

Answer 5

Multiple types, asymmetric hypertrophy of myocardial septum is commonest in exam.
Subset associated with LVOT (hypertrophic Obstructive cardiomyopathy).

Question 6

Hypertrophic cardiomyopathy - imaging (2)

Answer 6

HOCM: Anterior leaflet of mitral valve pulled into LVOT (Systolic Anterior Motion (SAM) of mitral valve)
Patchy, midwall delayed enhancement of hypertrophic muscle.

Question 7

Noncompaction - definition/trivia (3)

Answer 7

Rare, genetic cardiomyopathy.
Loosely packed myocardial fibres.
Heart failure at young age

Question 8

Noncompaction - imaging (2)

Answer 8

LV spongey appearance, increased trabeculations and deep intertrabecular recesses.
Defined as ratio of noncompacted to compacted myocardium of >2.3:1 at end diastole.

Question 9

Muscular dystrophy - definition/trivia (2)

Answer 9

X-linked.
Biventricular replacement of myocardium with connective tissue and fat

Question 10

Muscular dystrophy - types (2)

Answer 10

Becker (mild), Duchenne (severe)

Question 11

Muscular dystrophy - imaging (2)

Answer 11

Delayed enhancement of midwall.
Often also dilated cardiomyopathy.