Outcomes of Rehabilitation Strategies for Pulmonary Atresia with Ventricular Septal Defect: A Single Center's Experience

Background: Both systemic -to -pulmonary shunt and right ventricle -pulmonary artery (RV -PA) connection are extensively applied to initially rehabilitate the pulmonary artery in pulmonary atresia with the ventricle septal defect (PA/VSD). However, which of these options is the most ideal for promoting pulmonary artery development and improving outcomes remains controversial. Methods: A total of 109 PA/VSD patients undergoing initial rehabilitative surgery at Guangdong Provincial People's Hospital from 2010 to 2020 were enrolled in this study. A series of clinical data were collected to compare the perioperative and postoperative outcomes between systemic -to -pulmonary and RV -PA connection. Results: The mean duration of follow-up was 61.1 months in the systemic -to -pulmonary shunt group and 70.3 months in the RV -PA connection group (p > 0.05). The RV -PA connection technique resulted in a significantly higher PaO2, lower red blood cells (RBC), lower hemoglobin, and lower hematocrit (Hct) (p < 0.05). The cumulative incidence curve estimated a cumulative complete repair rate of 56 +/- 7% after 5 years in the RV -PA connection group, significantly higher than 36 +/- 7% after 5 years in the systemic -to -pulmonary shunt group (p < 0.05). The Kaplan -Meier curve revealed a similar estimated survival rate between the two groups (p = 0.73). The RV -PA connection was identified as an independent predictor for complete repair in the multivariable analysis (HR = 2.348, 95% CI = 1.131-4.873). Conclusions: The RV -PA connection is a more ideal initial rehabilitative technique than systemic -to -pulmonary shunt in treating PA/VSD as a consequence of comparable probability of survival but improved definitive complete repair rate.