Noncompaction of ventricular myocardium.

Noncompaction of ventricular myocardium is a rare cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium; its true prevalence is still uncertain. A strong association between noncompaction and neuromuscular disorders has been described. Its major clinical manifestations are heart failure, arrhythmias and systemic embolic events. Diagnosis of noncompaction of ventricular myocardium can be made by conventional two-dimensional and color Doppler echocardiography. Recently contrast echocardiography has been used to provide a definitive diagnosis in most cases in which conventional echocardiography could not do so. We describe the case of a patient with clinical suspicion of noncompaction of ventricular myocardium established by conventional two-dimensional and color Doppler echocardiography and definitive diagnosis, as well as additional information, obtained by cardiac magnetic resonance imaging. We also discuss the difficulties in defining the therapeutic approach.