Left Ventricular Noncompaction Cardiomyopathy (LVNC) is a rare condition affecting the cardiac muscles, characterized by a hypertrophic left ventricle with deep trabeculations, and systolic and diastolic dysfunction. The myocardium of the left ventricle appears spongy and non-compacted, and made up of a meshwork of muscle bands. Most affected patients present in infancy or early childhood with tachypnea caused by a low cardiac output. Some may also present with cyanosis, syncope, dysmorphic features, and a failure to thrive. The condition is rare, with an estimated incidence of 0.81 per 100,000 infants.
Diagnosis of LVNC usually relies on ECG, Doppler imaging, and/or cardiac MRI. Diagnostic criteria include the presence of multiple echocardiographic trabeculations, multiple deep intertrabecular recesses communicating with the ventricular cavity, and most importantly, increased non-compacted to compacted ratio, assessed by comparing the size of the muscle bands with the compacted muscles in the heart. The ECG may show evidence of marked biventricular hypertrophy, and features typical of Pompe's disease and Wolf-Parkinson-White syndrome. Differential diagnosis includes hypertrophic or dilated cardiomyopathy. Relevant treatment depends upon the clinical feature of the patient. The most important aspect, however, is the early detection of the abnormality, and prevention of further complications. Regular medications and treatment regimes used in heart failure can be used effectively for the management of LVNC too.