Outcomes of total anomalous pulmonary venous drainage repair in neonates with univentricular circulation

Survival after surgery for isolated total anomalous pulmonary venous drainage has improved in the current era. However, outcomes of total anomalous pulmonary venous drainage repair in patients with univentricular circulation are associated with high mortality. Furthermore, outcomes of surgery in neonates with univentricular circulation are rarely reported among larger studies. Hence, we reviewed our experience (n = 19) with this population at a single institution (between 1986 and 2015) to identify specific subgroups with high mortality. We found patients with right ventricular dominance had the highest early mortality (54%; 7 of 13) when compared with patients (17%; 1 of 6) with left ventricle and biventricular dominance. Five patients (26%; 5 of 19) underwent reoperation for postoperative pulmonary venous obstruction. There were 5 long-term survivors. In conclusion, total anomalous pulmonary venous drainage repair in neonates with univentricular circulation is rare and is associated with high mortality and reoperation.