Isolated left ventricular non-compaction cardiomyopathy in adults

Left ventricular non-compaction (LVNC) is a heart-muscle disorder characterized by prominent myocardial trabeculations and deep intertrabecular recesses in the LV cavity. LVNC is often diagnosed by echocardiography and cardiac magnetic resonance imaging, but a universally accepted definition of LVNC is lacking. Although the prevalence of LVNC in adults remains unclear, improvements in diagnostic techniques account for the relatively high incidence of LVNC in recent years. The clinical presentation is highly variable from asymptomatic to symptomatic. Meanwhile, the classical triad of heart failure, ventricular arrhythmias, and systemic embolism constitute typical complications of this disease. Unfortunately, there is no specific therapy for LVNC, and management depends on the clinical manifestations. In this review, we discuss what is currently known about LVNC and conclude that multicenter registries are required for a better understanding of this rare disorder. (C) 2014 Published by Elsevier Ltd on behalf of Japanese College of Cardiology.