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Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE).Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 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 models.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, stroke occurred in 16 (6%), and 23 (8%) died. The presence of 2 major coronary artery stenoses (adjusted odds ratio, 4.99; 95% CI, 1.16-21.39) and lower weight at first intervention (adjusted odds ratio, 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).In a multicenter cohort, 1 in 5 neonates with PA-IVS experienced MACE after their initial intervention. Patients with 2 major coronary artery stenoses or lower weight at the time of the initial procedure were most likely to experience MACE and warrant vigilance during preintervention planning and postintervention management.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was - 3.06 (25%, 75%: - 4.00, - 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29-0.75, p 0.001) and OR = 3.75 (95% CI 1.46-9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

Introduction: Optimal management of neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) with confluent pulmonary arteries is unknown. We sought to compare outcomes for patients who underwent primary complete repair vs. initial surgical palliation followed by delayed repair. Methods: We conducted a retrospective study at 20 centers within CoRe-PCICS (Collaborative Research from the Pediatric Cardiac Intensive Care Society). Data were collected on infants undergoing initial surgical intervention at 0 - 60 days of age with TOF/PA from 2009 to 2018, excluding patients with MAPCAs or those undergoing ductal stenting (n=22). The primary outcome was days alive and out of hospital in first year of life (DAOH). Secondary outcomes were 1 year mortality and a composite major complication outcome (similar to that in prior STS-CHSD studies), defined as occurrence of ≥ 1 of the following: renal failure requiring dialysis, stroke/seizure, permanent pacemaker, ECMO, or diaphragm paralysis during a palliation and/or repair hospitalization, or unplanned reoperation in the first year. Multivariable modeling with generalized estimating equations were utilized to compare outcomes between groups. Results: Of 210 subjects, 79 underwent primary complete repair and 131 underwent surgical palliation. Patients who underwent palliation had greater use of preoperative mechanical ventilation at first procedure (26% vs. 8%, p = 0.002). Other baseline characteristics were similar between groups (p > 0.05 for all). There was no statistically significant difference in DAOH between the palliation and primary repair groups [median (25%,75% IQR): 319 (280,336) vs. 338 (314,348 days), adjusted p = 0.20]. Nine (7%) patients who underwent palliation died in the first year of life vs. 4 (6 %) who underwent primary repair (adjusted OR: 1.1, 95% CI: 0.3-4.5; p = 0.9). At least one major complication occurred in 35% of patients who underwent palliation vs. 18% of patients who underwent primary repair (adjusted OR: 2.5, 95% CI: 1.4-4.4, p = 0.001). Conclusions: For infants with TOF/PA with confluent pulmonary arteries, a strategy of surgical palliation or primary complete repair resulted in similar DAOH and early mortality, whereas the morbidity incidence favored primary repair.