South African Journal of Child Health
On-line version ISSN 1994-3032
ABSTRACT Isolated left ventricular non-compaction (LVNC) is a rare congenital disorder caused by failure of normal embryogenesis of the endocardium and myocardium in utero, resulting in prominent ventricular trabeculations with deep intertrabecular recesses. Patients may be asymptomatic or may present with refractory cardiac failure. The best modality of diagnosis is based on echocardiographic diagnostic criteria. Prognosis remains poor for patients with impaired systolic left ventricular function, as treatment options are very limited. We report the case of 2-month-old infant presenting with congestive cardiac failure diagnosed as isolated LVNC on echocardiography. Left ventricular non-compaction (LVNC) is a congenital dysfunction of ventricular morphogenesis. The World Health Organization has categorised it as an unclassified cardiomyopathy. It is thought to be the result of an arrest in the normal endomyocardial embryogenesis, which leads to the persistence of intertrabecular recesses and the development of a spongy myocardium. LVNC is now being diagnosed with increasing frequency, either in isolation or combined with congenital heart disease or neuromuscular disorders. Here we report the case of a 2-month-old male infant who presented with congestive heart failure (HF) where isolated LVNC was found on echocardiography.