Primary Versus Staged Repair in Neonates with Pulmonary Atresia and Ventricular Septal Defect

Abstract Background The two surgical strategies for neonates with ductal-dependent pulmonary atresia and ventricular septal defect (PA VSD) are primary biventricular repair (BVR) or initial palliation with a modified Blalock-Taussig shunt (BTS) followed by second stage repair. In this study, we report the combined outcomes from 2 hospitals using different strategies. Methods Between 2004 and 2017, 66 neonates underwent surgery with palliative shunts (BTS group: n=30, 45.5%) or primary biventricular repair (pBVR group: n=36, 54.5%). The two groups were similar in age, body weight, and Nakata index scores. The overall mean follow-up duration was 7.51 ± 4.35 years, and early and late results were compared between the groups. Results The 10-year overall survival was 84.8% (94.4% for pBVR versus 75.7% for BTS, p=0.032). The BTS group had 2 early and 6 interstage mortalities, while the pBVR group had no early and 2 late mortalities. In the BTS group, the Nakata index score significantly increased during the interstage period (p Conclusions In neonates with ductal-dependent PA VSD, the primary BVR approach provides an excellent survival rate, but the burden of RVOT reintervention is very heavy. The staged approach with BTS promotes pulmonary artery growth, but hospital and interstage mortality are significant. Genetic and extracardiac anomalies are significant risk factors for mortality.