Procedural outcomes of pulmonary atresia with intact ventricular septum in neonates: A multicenter study

Background: Multicenter contemporary data describing short-term outcomes following initial interventions of neonates with pulmonary atresia intact ventricular septum (PA-IVS) are limited. This multicenter study aims to describe characteristics and outcomes of PA-IVS neonates following their initial catheter or surgical intervention and identify factors associated with major adverse cardiac events (MACE). Methods: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009-2019 in 19 centers were reviewed. Risk factors for MACE, defined as cardiopulmonary resuscitation, mechanical circulatory support, stroke, or in-hospital mortality, were analyzed using multivariable logistic regression model. Results: We reviewed 279 neonates: 79 (28%) underwent right ventricular decompression, 151 (54%) underwent systemic-to-pulmonary shunt or ductal stent placement only, 36 (13%) underwent right ventricular decompression with shunt or ductal stent placement, and 11 (4%) underwent transplantation. MACE occurred in 57 patients (20%): 26 (9%) received mechanical circulatory support, 37 (13%) received cardiopulmonary resuscitation, 16 (6%) suffered stroke, 23 (8%) died. The presence of two major coronary artery stenoses (adjusted OR: 4.99; 95% CI: 1.16-21.39) and lower weight at first intervention (adjusted OR: 1.52, 95% CI: 1.01-2.27) were significantly associated with MACE. Coronary ischemia was the most frequent presumed mechanism of death (n=10). Conclusions: In a multicenter cohort, one in five neonates with PA-IVS experienced MACE following their initial intervention. Patients with two major coronary artery stenoses or lower weight at time of initial procedure were most likely to experience MACE and warrant vigilance during pre-intervention planning and post-intervention management.