[Isolated non-compaction of left ventricular myocardium]

Noncompaction of the ventricular myocardium, also known as "spongy myocardium", is a rare congenital abnormality resulting from an arrest in the normal endomyocardial embryogenesis. The echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intratrabecular recesses in communication with the left ventricular cavity. This entity is a not well known cause of dilated cardiomyopathy. Some cases were described as X-linked familial forms. We report the clinical case of a 13-year-old female patient with severe left ventricular disfunction, a very trabeculated left ventricle on echocardiography and two admissions in class IV heart failure.