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Background: Few patients with total anomalous pulmonary venous drainage (TAPVD) and a univentricular circulation survive to Fontan completion. Hence, we sought to determine the long-term outcomes of the Fontan operation in patients with TAPVD., Methods: Patients with TAPVD who underwent the Fontan operation and survived to hospital discharge in Australia and New Zealand between 1985 to 2017 were identified (n = 54) from a binational Fontan registry., Results: Thirty-two patients (60%) underwent repair of TAPVD at a median age of 0.8 (interquartile range: 0.3-1.6) years. Thirty-seven patients (69%) had heterotaxy. The median age at time of Fontan operation was 5.7 years. There were 4 late deaths and 3 patients required cardiac transplantation for a failing Fontan circulation. On univariate analysis, the concomitant diagnosis of pulmonary stenosis and right ventricular dominance was associated with late death or transplantation (P = .04). Freedom from late death or transplantation at 15 years after the Fontan operation was 88% ± 7% (95% confidence interval [CI], 67%-96%) for the repaired TAPVD group and 90% ± 6% (95% CI, 67%-98%) for the unrepaired TAPVD group (P = .47). Median follow-up after the Fontan procedure was 10.8 (interquartile range, 6.7-16.2) years. The majority of survivors (94%) were in New York Heart Association functional class I or II. The 15-year freedom from death or transplantation was similar for patients with TAPVD (89% ± 5%; 95% CI, 76%-95%) compared with patients without TAPVD in the Fontan registry (n = 1446; 92% ± 1%; 95% CI, 90%-93%) (P = .12)., Conclusions: Long-term survival of patients with TAPVD who undergo the Fontan operation and survived to hospital discharge is comparable to Fontan survivors without TAPVD., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.