RESUMO
Isolated left ventricular noncompaction is a rare congenital cardiomyopathy, characterized morphologically by a dilated left ventricle, prominent trabeculations and deep intertrabecular recesses in the ventricular myocardium, with no other structural heart disease. It is thought to be secondary to an arrest of normal myocardial compaction during fetal life. Clinically, the disease presents with heart failure, embolic events, arrhythmias or sudden death. Current diagnostic criteria are based on clinical and imaging data and two-dimensional and color Doppler echocardiography is the first-line exam. There is no specific therapy and treatment is aimed at associated comorbidities. Cases refractory to medical therapy may require heart transplantation. The authors describe a case of severe and refractory heart failure, which was the initial presentation of isolated left ventricular noncompaction in a previously healthy male child, who underwent successful heart transplantation.