Abstract:
Left ventricular noncompaction has been classified as a primary cardiomyopathy with a genetic origin. This condition is
morphologically
characterized by a thickened, two-layered myocardium with numerous prominent trabeculations and deep, intertrabecular
recesses. Recently, it has become clear that these pathological characteristics extend across a continuum with left ventricular
hypertrabeculation at one end of the spectrum.
The histological findings include areas of interstitial fibrosis.
We present a case of left ventricular hypertrabeculation which presented as sudden infant death syndrome. Histologically areas of subendocardial
fibrosis was prominent and we propose that this entity may be a hidden cause of arrhythmic death in some infants presenting
as sudden infant death syndrome., with areas of subendocardial fibrosis as possible arrhythmogenic foci.
