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Background: We sought to determine the long-term quality of life after repair of anomalous pulmonary venous drainage using the Short Form (SF)-36 questionnaire in adult survivors., Methods: All patients who underwent repair of partial or total anomalous pulmonary venous drainage (PAPVD or TAPVD) and were 18 years of age or older with a current contact number were identified from the hospital database. The mean age of the 101 patients was 26 ± 7 years (range, 18-49) old. Patients completed the SF-36 quality of life questionnaire via telephone. The results of the 8 domains of the SF-36 questionnaire and the derived health state summary score (SF-6-Dimension) were compared against an age-matched Australian population data., Results: Compared with Australian population age-matched data, the 18- to 24-year-old TAPVD/PAPVD patients ranked their health higher in 1 of 8 domains; however the SF-6-Dimension scores were similar (0.75 for TAPVD and PAPVD patients vs 0.77 for the Australian population, P = .2). In the 25-50 age group TAPVD/PAPVD patients ranked their health higher in 3 of 8 domains. However the SF-6-Dimension scores were similar to Australian age-matched population (0.78 for TAPVD and PAPVD patients vs 0.77 for the Australian population, P = .51)., Conclusions: Young adult survivors after anomalous pulmonary venous drainage repair have similar quality of life outcomes as age-matched Australian control subjects as measured by SF-6-Dimension., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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.)