Long-term Outcomes of the Fontan Operation in Patients With Total Anomalous Pulmonary Venous Drainage.

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.