Intrinsic obstruction in pulmonary venous drainage pathway is associated with poor surgical outcomes in patients with total anomalous pulmonary venous connection.

The aim of this study was to review the surgical outcomes of conventional repair in patients with total anomalous pulmonary venous connection (TAPVC). Between 1999 and 2012, 46 patients underwent conventional TAPVC repair; of those, 14 required emergent surgery within the first 24 h after their birth for coexisting intrinsic obstruction in pulmonary venous drainage pathway (Group 1). The remaining 32 were operated on after the first day of life and were divided into two groups: with (Group 2, n = 10) and without (Group 3, n = 22) subsequently progressed pulmonary venous obstruction (PVO) after birth. A follow-up was completed on all patients, and the mean follow-up period was 7 ± 4 years (range 0.9-14). Group 1 required a significantly prolonged nitric oxide inhalation (12 ± 11 days) and mechanical ventilation support (29 ± 36 days) compared to Group 2 and Group 3. The actuarial survival rate at 10 years was 69% in Group 1, 88% in Group 2, and 96% in Group 3 (Group 1 vs. Group 3: p = 0.05). Freedom from postoperative pulmonary vein stenosis at 10 years was 39% in Group 1, 70% in Group 2, and 86% in Group 3 (Group 1 vs. Group 3: p = 0.002). However, all the ten survivors in Group 1 showed an NYHA functional status of I or II, and 60% of survivors were free from medication. Outcomes of patients with TAPVC requiring surgical repair at the day of birth for coexisting intrinsic PVO were still dismal; however, the acceptable status of current survivors has encouraged us to treat this challenging group surgically.