Isolated Noncompaction of the Ventricular Myocardium

Synonyms

Isolated Non-compaction of the Ventricular Myocardium

Isolated Noncompaction of the Left Ventricular Myocardium, X-Linked

Noncompaction of the Left Ventricular Myocardium, Autosomal Dominant

Rare congenital cardiomyopathies characterized by the lack of left ventricular myocardium compaction. The noncompaction results in numerous prominent trabeculations and a loose myocardial meshwork (spongy myocardium) in the LEFT VENTRICLE. Heterogeneous clinical features include diminished systolic function sometimes associated with left ventricular dilation, that presents either neonatally or progressively. Often, the RIGHT VENTRICLE is also affected. CONGESTIVE HEART FAILURE; PULMONARY EMBOLISM; and ventricular ARRHYTHMIA are commonly seen.