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

The 2 surgical strategies for neonates with ductal-dependent pulmonary atresia and ventricular septal defect 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.

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 2 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.

The 10-year overall survival was 84.8% (94.4% for pBVR vs 75.7% for BTS, P = .032). The BTS group had 2 early and 6 interstage mortalities, and 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 < .001). In univariable analysis, genetic or extracardiac anomalies were a risk factor for mortality (hazard ratio, 5.56; P = .038). After achieving BVR, the pBVR group underwent significantly more frequent right ventricle outflow tract reinterventions (P < .001) at a much earlier period (P = .017) compared with the BTS group.

In neonates with ductal-dependent pulmonary atresia and ventricular septal defect, the primary BVR approach provides an excellent survival rate, but the burden of right ventricle outflow tract reintervention is 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.