Left ventricular noncompaction cardiomyopathy: what do we know?

Noncompaction is a rare and primary genetic cardiomyopathy affecting the left ventricle. The diagnosis is usually established by echocardiography, or, less frequently, by left ventriculography. Cardiac magnetic resonance and electrocardiography-gated multi-detector computed tomography are evolving noninvasive modalities to image cardiac structures, and have the utility to detect noncompacted myocardium. Estimates of the frequency and incidence of left ventricular noncompaction (LVNC) are limited because of the controversy over whether LVNC is a discrete disease entity. There is considerable overlap with dilated cardiomyopathy, apical hypertrophy, and hypertrophic cardiomyopathy. Symptoms, diagnosis, and prognosis are variable because of the heterogeneous nature of these diseases, making treatment often empirical and mimicking the treatment of other cardiomyopathies. However, there are management issues that should be addressed in each patient with LVNC, including genetic testing and family screening, the need for implantable cardioverter defibrillator placement, the role of anticoagulation in prevention of thromboembolic complications, and prescriptions/restrictions for implementation of physical activity.