Isolated left ventricular noncompaction: a review of three different presentations for a rare disease.

Isolated left ventricular noncompaction (ILVNC) is a rare cardiomyopathy with a genetic background characterized by numerous prominent trabeculations and deep intertrabecular recesses. It occurs in the absence of any coexisting congenital lesion or hemodynamic abnormality and is rare in the adult population. Heart failure, ventricular arrhythmias, and embolic events are the three major clinical manifestations of ILVNC. Medical and surgical treatment is similar to other systolic dysfunction cardiomyopathies and depends on the presenting clinical manifestations. In this review, we present three patients with different clinical presentations of ILVNC leading to different treatment modalities ranging from medications alone to device implantation and transplantation. Pathologic findings from one of our patients are also presented.