Left ventricular noncompaction cardiomyopathy
Sherling, K.W.; Nuttli, T.; Newman, W.P.; McGoey, R.R.
Journal of the Louisiana State Medical Society Official Organ of the Louisiana State Medical Society 165(3): 150-152
2013
ISSN/ISBN: 0024-6921 PMID: 24015429 Document Number: 668214
Isolated Left Ventricular Non Compaction Cardiomyopathy (LVNCC) is a rare genetic cardiomyopathy characterized by a thickened left ventricle with two distinct layers - an inner noncompacted (spongy) layer and an outer compact (dense) layer. The spongy layer is composed of deep intertrabecular recesses. Familial and sporadic forms are known to exist and there is significant genetic heterogeneity among the inherited forms. Symptoms vary and range from asymptomatic to severe heart failure and sudden death. The clinical diagnosis of LVNCC can be challenging partly because there is currently no consensus for diagnostic criteria. As such, many affected individuals are, inopportunely, first diagnosed at post-mortem examination.