Familial isolated noncompaction of the left ventricular myocardium

Noncompaction of the ventricular myocardium (sometimes referred to as 'spon gy myocardium') is believed to represent an arrest in endomyocardial morpho genesis. The gross anatomical appearance is characterized by numerous exces sively prominent trabeculations and deep intertrabecular recesses. Distinct morphological features can be diagnosed on two-dimensional echocardiograph y, We present here a family of isolated noncompaction of the left ventricul ar myocardium, in which 5 affected individuals suggested the presence of so me genetic abnormalities in this disorder.