Long-term Outcomes of Children Operated on for Anomalous Left Coronary Artery From the Pulmonary Artery

• Type of Material: Article
• Authors: Amanda S Thomas, Emory University; Alice Chan, Duke University; Bahaaldin Alsoufi, Emory University; Jeffrey M Vinocur, University of Rochester School of Medicine and Dentistry; Lazaros Kochilas, Emory University
• Language: English
• Date: 2022-03-25
• Publisher: ELSEVIER SCIENCE INC
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2022 by The Society of Thoracic Surgeons
• Final Published Version (URL): http://dx.doi.org/10.1016/j.athoracsur.2021.07.053
• Title of Journal or Parent Work: ANNALS OF THORACIC SURGERY
• Volume: 113
• Issue: 4
• Start Page: 1223
• End Page: 1230
• Keywords: COMPLICATIONS; Cardiovascular System & Cardiology; Respiratory System; Surgery; Cardiac & Cardiovascular Systems; LEFT-VENTRICULAR DYSFUNCTION; Science & Technology; RECOVERY; TAKEUCHI REPAIR; SURGICAL INTERVENTION; Life Sciences & Biomedicine; ORIGIN; INFANTS; SURVIVAL

Abstract

Background: We examined the outcomes of children (<18 years) operated on for anomalous left coronary artery from the pulmonary artery (ALCAPA). Methods: We linked patients undergoing ALCAPA repair between 1982 and 2003 in the Pediatric Cardiac Care Consortium with the National Death Index and the Organ Procurement and Transplantation Network to examine their outcomes through 2019. Results: We identified 228 children (median age, 6.0 months) operated on for ALCAPA. At the time of repair, 38.6% had severe mitral regurgitation (MR), and 71.4% had severe left ventricular (LV) dysfunction. Repair included primarily coronary reimplantation in 173 and the Takeuchi procedure in 34; concurrently, 18 underwent mitral valve (MV) operation. In-hospital death occurred in 31 (13.6%) and was not associated with MR severity (P = .846); however, among patients with moderate or severe MR, risk of death was 28% lower when undergoing MV operation (P = .033). After adjustment for other risk factors, only infant operation reached statistical significance for in-hospital death (adjusted odds ratio, 12.99; 95% CI, 1.61-104.59; P = .016). Among those discharged alive with long-term data available (n = 155), the 30-year transplant-free survival reached 95.5% (95% CI, 92.3%-98.8%) and was not associated with the degree of preoperative MR or LV dysfunction. Coronary reimplantation was associated with better long-term survival compared with other surgical techniques (adjusted odds ratio, 0.11; 95% CI, 0.02-0.74; P = .023). Conclusions: Favorable long-term outcomes can be expected after coronary artery reimplantation for ALCAPA, even in patients with severe LV dysfunction at presentation. MV operation predicted decreased risk for in-hospital mortality in patients with moderate/severe MR, but MR severity predicted neither in-hospital nor longer-term outcomes.