2023 Volume 7 Pages 66-74
Background: Heterotaxy syndrome associated with functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC) is characterized by high operative risks and has high mortality rate. Treatment strategies to address these risks remain uncertain.
Methods: This retrospective study included 16 patients with heterotaxy syndrome and functional single ventricle and TAPVC who underwent TAPVC repair between 2003 and 2023. The patients were divided into two groups according to the time of TAPVC repair and the type of repair technique Used. The actuarial survival and freedom from reoperation for pulmonary venous obstruction were compared.
Results: Actuarial survival differed significantly between the two groups (early group: 38.5% vs. late group: 100% at 1 year, p < 0.05). A comparison of TAPVC repair techniques revealed higher freedom from reoperation for pulmonary venous obstruction in the primary sutureless technique group (n = 6) than in the conventional repair technique group (100% vs. 63 % at 1 year, p = 0.15).
Conclusion: Improved surgical outcomes by delaying surgical TAPVC repair and using primary sutureless technique for initial TAPVC repair, if possible.
