/left ventricular noncompaction

left ventricular noncompaction

Rare Disease

MONDO:0018901

Also Known As

LVNCLv non-compaction syndromeleft ventricular hypertrabeculationleft ventricular non-compaction cardiomyopathyleft ventricular non-compaction syndromeleft ventricular noncompaction (disease)spongy myocardium

Definition

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy characterized anatomically by prominent left ventricular trabeculae and deep intratrabecular recesses causing progressive systolic and diastolic dysfunction, conduction abnormalities, and occasionally thromboembolic events.