Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA): A 15-Year Retrospective Cohort Study at a Tertiary
Vítor Doria Ricardo1, Jessica Fonseca Campos de Oliveira2, Felipe Yu Matsushita2
1Instituto do Coração (InCor), Hospital das Clínicas, Faculdade de Medicina Universidade de São Paulo - Pediatric Cardiology Department, Av. Dr. Enéas de Carvalho Aguiar, 44, São Paulo, SP, 05403-900, Brazil. vitordoria@hotmail.com.
Abstract:
Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) is a rare congenital coronary anomaly associated with high mortality without surgical correction. Long-term outcome data from Latin America remain limited. To describe the clinical characteristics, surgical management, and postoperative outcomes of patients with ALCAPA treated over a 15-year period at a tertiary referral center in Brazil. This retrospective cohort study included 71 patients with confirmed ALCAPA whose institutional records were reviewed from January 2010 through December 2024. Clinical characteristics, surgical procedures, echocardiographic findings, perioperative mechanical circulatory support, follow-up duration, reoperations, transplantation, and mortality were analyzed using descriptive and exploratory statistical methods. The cohort comprised 71 patients (56.3% female), with a median age at surgery of 6 months. Preoperative left ventricular dysfunction and/or mitral regurgitation was present in 38.0% of patients. Median postoperative follow-up was 9.0 years. Overall mortality was 5.6%, including three deaths within the first year of life. Reoperation was required in 6 of 69 surgically treated patients (8.7%). Five patients (7.2%) required perioperative extracorporeal membrane oxygenation, with one death. Among patients with preoperative dysfunction, 59.2% demonstrated postoperative echocardiographic recovery. Surgical correction of ALCAPA was associated with low mortality and substantial recovery of ventricular function. These findings reinforce the importance of early diagnosis, prompt referral, and long-term follow-up at specialized centers.
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