Left ventricular noncompaction: a 25-year odyssey

Abstract

Left ventricular noncompaction (LVNC) is a cardiomyopathy associated with sporadic or familial disease, the latter having an autosomal dominant mode of transmission. The clinical features associated with LVNC vary from asymptomatic to symptomatic patients, with the potential for heart failure, supraventricular and ventricular arrhythmias, thromboembolic events, and sudden cardiac death. Echocardiography is the diagnostic modality of choice, revealing the pathognomonic features of a thick, bilayered myocardium; prominent ventricular trabeculations; and deep intertrabecular recesses. Widespread use and advances in the technology of echocardiography and cardiac magnetic resonance imaging are increasing awareness of LVNC, and cardiac magnetic resonance imaging is improving the ability to stage the severity of the disease and potential for adverse clinical consequences. Study of LVNC through research in embryology, imaging, and genetics has allowed enormous strides in the understanding of this heterogeneous disease over the past 25 years.