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

• Published in: Circulation (New York, N.Y.) Vol. 144; no. Suppl_1; p. A9472
• Main Authors: Boucek, Katerina, Mastropietro, Christopher, Beall, Jonathan, Keller, Everette, Beshish, Asaad, Flores, Saul, Chlebowski, Meghan, Yates, Andrew R, Choudhury, Tarif, Mueller, Dana, Kwiatkowski, David M, Migally, Karl, Karki, Karan, Willett, Renee, Radman, Monique R, Reddy, Chetana, Piggott, Kurt, Capone, Christine, Kapileshwarkar, Yamini, Vijayakumar, Niranjan, Prentice, Elizabeth, Narasimhulu, Sukumar Suguna, Martin, Renee H, Costello, John M
• Format: Journal Article
• Language: English
• Published: Lippincott Williams & Wilkins 16.11.2021
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/circ.144.suppl_1.9472

IntroductionOptimal 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. MethodsWe 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 followingrenal 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. ResultsOf 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 OR1.1, 95% CI0.3-4.5; 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 OR2.5, 95% CI1.4-4.4, p = 0.001). ConclusionsFor 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.