Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database

Abstract

Introduction We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. Materials and Methods From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in‐hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). Results The overall in‐hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross‐clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in‐hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). Conclusions ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

Case Report: Neonatal Complex Congenital Heart Disease With Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery: Analysis of Missed Diagnosis and Improvement Procedures

“…If severe left ventricular dysfunction occurs after the operation, it is necessary to be vigilant and look for evidence of the ALCAPA. An exploration of the coronary artery is mainly recommended for children with complex CHD in the neonatal period and children with CHD with unexplained cardiac insufficiency and abnormal mitral valve development, which may be indirect signs of the ALCAPA (1,7,8). The above improvement procedures are strongly recommended in neonatal complex CHD surgery, which can avoid the missing diagnosis of the ALCAPA.…”

“…The anomalous origin of the left coronary artery from the right pulmonary artery (ALCAPA) is a rare type of congenital disease. Combined with complex congenital heart diseases (CHDs) requiring surgical intervention in the neonatal period, it is even rarer (1)(2)(3). We report a neonate with complex CHD.…”

Pulmonary artery banding as adjunct therapy for ventricular recovery after anomalous left coronary artery from the pulmonary artery repair: a case report

“…A recent publication from the European Congenital Heart Surgeons Association Database reports on an in-hospital mortality of 6% (out of 907 patients undergoing ALCAPA repair between 1999-2019) [7]. In this cohort MCS was required in 7.3% of patients, almost exclusively in infants, resulting in a signi cantly higher mortality [7].…”

“…Currently, surgical correction aims to restore antegrade coronary perfusion by direct coronary reimplantation or intrapulmonary tunnel-type repair after which spontaneous and progressive myocardial recovery is observed in most cases [1,6]. However in some patients temporary post-operative mechanical circulatory support (MCS) -either by extracorporeal membrane oxygenation (ECMO) or left ventricle assist devices (LVAD) -is required to allow myocardial recovery (CPB) [7]. A recent publication from the European Congenital Heart Surgeons Association Database reports on an in-hospital mortality of 6% (out of 907 patients undergoing ALCAPA repair between 1999-2019) [7].…”

“…However in some patients temporary post-operative mechanical circulatory support (MCS) -either by extracorporeal membrane oxygenation (ECMO) or left ventricle assist devices (LVAD) -is required to allow myocardial recovery (CPB) [7]. A recent publication from the European Congenital Heart Surgeons Association Database reports on an in-hospital mortality of 6% (out of 907 patients undergoing ALCAPA repair between 1999-2019) [7]. In this cohort MCS was required in 7.3% of patients, almost exclusively in infants, resulting in a signi cantly higher mortality [7].…”