Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

Lee WY, Kang SR, Im YM, Yun TJ.Pediatr Cardiol. 2020 May 6:1-9. doi: 10.1007/s00246-020-02352-9. Online ahead of print.PMID: 32377890 Free PMC article.



Background: The optimal surgical strategy for pulmonary atresia with ventricular septal defect (PA/VSD) in neonates and young infants is controversial. Staged repair may be associated with a higher risk of inter-stage mortality, while primary repair may lead to frequent post-repair re-interventions.

Methods: From 2004 to 2017, 65 patients with PA/VSD who underwent surgical intervention before 90 days of age were identified and enrolled in this retrospective study. The cohort was divided into two groups: group-SR, who underwent initial palliation with staged repair (n = 50), and group-PR who underwent primary repair (n = 15).

Results: There were three post-palliation in-hospital mortalities, four inter-stage mortalities, and one post-repair in-hospital mortality in group-SR. In group-PR, there was one in-hospital death and one late death. Five-year survival rates were comparable between the two groups (group-SR: 83.6%; group-PR: 86.7%; p = 0.754). During the median follow-up duration of 44.7 months (Inter-quartile range, 19-109 months), 40 post-repair re-interventions (22 in group-SR, 18 in group-PR) were performed in 26 patients (18 in group-SR, 8 in group-PR). On Cox proportional hazards model, primary repair was identified as the only risk factor for decreased time to death/1st post-repair re-intervention (Hazard ratio (HR): 2.3, p = 0.049) and death/2nd post-repair re-intervention (HR 2.91, p = 0.033).

Conclusions: A staged repair strategy, compared with primary repair, was associated with comparable overall survival with less frequent re-interventions after repair in young infants with PA/VSD. Lowering the inter-stage mortality after initial palliation by vigilant outpatient care and aggressive home monitoring may be the key to better surgical outcomes in this subset. Surgical outcomes of PA with VSD according to the surgical strategies. Patient 1 (birth weight: 2.7 kg) underwent primary Rastelli-type repair at post-natal day # 50 (body weight: 3.8 kg) using Contegra® 12 mm. The postoperative course was rocky, with long ventilatory support (10 days), ICU stay (14 days), and hospital stay (20 days). Cardiac CT scan at 9 months post-repair showed severe branch pulmonary artery stenosis, which necessitated LPA stenting at 12 months post-repair and RV-PA conduit replacement with extensive pulmonary artery reconstruction at 25 months post-repair. Patient 2 (birth weight: 2.5 kg) underwent RMBT at post-natal day #30 (body weight: 3.4 kg) using 4 mm PTFE vascular graft and staged Rastelli-type repair at post-natal 11 months using a hand-made Gore-Tex valved conduit (14 mm). No post-repair re-intervention has been performed. Cardiac CT scan at 90 months post-repair showed no branch pulmonary artery stenosis.CT computed tomography, ICU intensive care unit, LPA left pulmonary artery, PA pulmonary atresia, PTFE polytetrafluoroethylene, RMBT right modified Blalock-Taussig shunt, RV-PA right ventricle to pulmonary artery, VSD ventricular septal defect.



Fig. 1 Outcomes of 65 patients with PA with VSD according to the surgical strategy. PA pulmonary atresia, VSD ventricular septal defect


Fig. 2 Post-natal survival in the two groups with different surgical strategies. PR primary repair, SR staged repair


Fig. 3 Freedom from death or 1st post-repair re-intervention in two groups with different surgical strategies. PR primary repair, SR staged repair


Fig. 4 Freedom from death or 2nd post-repair re-intervention in two groups with different surgical strategies. PR primary repair, SR staged repair


Fig. 5 Chronological plotting of death (red dots), 1st post-repair re-intervention (blue dots), and 2nd post-repair re-intervention (black dots) in two groups with different surgical strategies. PR primary repair, SR staged repair