Correction of anomalous systemic venous drainage in heterotaxy syndrome

A 3-month-old patient with heterotaxy syndrome, complex intracardiac malformations, and severe heart failure underwent surgical correction. Anatomy included an interrupted inferior vena cava with hemiazygous continuation to a persistent left superior vena cava draining to the left atrium. The presence of partial anomalous pulmonary venous drainage precluded an intraatrial baffle. Systemic venous reconstruction was achieved using the left atrial appendage along with an intracardiac repair of the other defects.