Successful palliation of a child with left ventricular noncompaction cardiomyopathy and tricuspid atresia to Fontan procedure.

A newborn baby girl was diagnosed with tricuspid atresia, ventricular septal defect, normally related great vessels, and hypoplastic right ventricle with no pulmonary stenosis. There was extensive left ventricular noncompaction cardiomyopathy. The left ventricular ejection fraction was 58%. She underwent placement of a pulmonary arterial band at 4 weeks of age, a bilateral bidirectional Glenn at 10 months of age, and fenestrated extracardiac total cavopulmonary connection at 3 years of age. The presence of left ventricular noncompaction in a patient with univentricular circulation does not necessarily circumvent successful cavopulmonary palliation, when left ventricular function is relatively preserved, as in this patient.