title: Left ventricular noncompaction cardiomyopathy.
abstract: 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.
authors.affiliation: Department of Pathology, Louisiana State University Health Sciences Center, New Orleans, USA.
authors.forename: Kelley Willis
authors.initials: KW
authors.lastname: Sherling
authors.forename: Theresa
authors.initials: T
authors.lastname: Nuttli
authors.forename: William P
authors.initials: WP
authors.lastname: Newman
authors.forename: Robin R
authors.initials: RR
authors.lastname: McGoey
mesh_terms: D042783:Endothelial Cells / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged