Ker J, Du Toit-Prinsloo L, Van Heerden W F P, Saayman G
Department of Physiology, University of Pretoria, Pretoria, South Africa.
Clin Med Insights Cardiol. 2011 Feb 2;5:13-6. doi: 10.4137/CMC.S6507.
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.
左心室心肌致密化不全已被归类为一种具有遗传起源的原发性心肌病。这种病症在形态学上的特征是心肌增厚,呈两层结构,有许多突出的肌小梁和深陷的小梁间隐窝。最近,已经明确这些病理特征在一个连续谱上延伸,一端是左心室肌小梁增多。组织学发现包括间质纤维化区域。我们报告一例以婴儿猝死综合征形式出现的左心室肌小梁增多病例。组织学上,心内膜下纤维化区域很突出,我们认为该实体可能是一些表现为婴儿猝死综合征的婴儿心律失常性死亡的潜在原因,心内膜下纤维化区域可能是致心律失常灶。