Familial isolated noncompaction of the left ventricular myocardium.

Noncompaction of the ventricular myocardium (sometimes referred to as 'spongy myocardium') is believed to represent an arrest in endomyocardial morphogenesis. The gross anatomical appearance is characterized by numerous excessively prominent trabeculations and deep intertrabecular recesses. Distinct morphological features can be diagnosed on two-dimensional echocardiography. We present here a family of isolated noncompaction of the left ventricular myocardium, in which 5 affected individuals suggested the presence of some genetic abnormalities in this disorder.