ECHO 2013 / Systolic Heart Failure Review of Cardiomyopathy

Hyperreflexive moderator band

Etiology of ARVD

•Pathogenesis unknown

•Often familial

•Several genes implicated

–Autosomal dominant

•7 loci mapped to chromosome 14

–Autosomal recessive

•Plakoglobin gene (cell-cell junctions)

–? Inadequate cell adherence → cell membrane damage → cell death + fibrofatty replacment

•Ryanodine receptor gene

–Induces Ca++ release from SR

–Adrenergically mediated arrhythmias

Echocardiography for ARVD

•Diastolic bulge and/or systolic dyskinesia of a thinned inferobasal wall adjacent to TV

•Hyper-reflective moderator band

•Isolated dilatation of the RVOT

•Abnormal RV trabecular pattern

–“trabecular disarray”

•Sacculations of the RV free wall and/or the anterior wall of the RVOT

•RV global dilatation

•Wall motion abnormalites

TT a 28 yo M with stroke

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