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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