Abstract 9472: Multicenter Comparison of Staged vs. Complete Repair in Young Infants With Tetralogy of Fallot with Pulmonary Atresia and Confluent Branch Pulmonary Arteries

Abstract

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.