Isolated noncompaction involving the left ventricular apex in adults

We present a series of 9 adults in 2 families with isolated noncompaction of the ventricular myocardium involving the left ventricular apex. Three of these patients had been previously misdiagnosed as having dilated cardiomyopathy, apical hypertrophic cardiomyopathy, or apical mural thrombus. Phenotypic variation was observed with gradation of severity of noncompaction and left ventricular systolic dysfunction between first-degree relatives.