ECHO 2013 / Systolic Heart Failure Review of Cardiomyopathy
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Myocardial biopsy
Apple red birefringence with polarizing light
•Young patient who faints while playing hockey
6 min on treadmill
Dilatation of RVOT
Dilated RV + RV dysfunction
Arrhythmogenic Right Ventricular dysplasia
ARVD
•Progressive replacement of RV myocardium by adipose and fibrous tissue
•“Triangle of dyplasia”
–RV infundibulum
–RV apex
–Inferior wall of RV
•Characterized by electrical instability due to reentry
•Important cause of sudden death in young people
•Diagnostic criteria proposed in 1994
Epidemiology
•Sex: Usually males
•Age: Late teens-mid 30s
•Symptomatic arrhythmia
–PVC s
–NSVT or sustained VT
•Often LBBB morphology
•Often asymptomatic until an episode of sudden cardiac death
–Young patient
–Exercise-induced
Increased trabeculations
Focal RV apical aneurysm
Thinning and bulging just below
TV posterior leaflet
