Long-Term Outcome of the Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery (ALCAPA) in Children After Cardiac Surgery: A Single-Center Experience.

Muna Ismail,Rawan Alshahrani,Rathath M Alhuwaymil,Reem Almutairi,Ghassan A Shaath,Abdulraouf Jijeh,Raneem Alahmari,Fahad Habshan

doi:10.7759/cureus.11829

Abstract

BackgroundThe anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery anomaly. It induces left ventricular (LV) dysfunction and mitral valve regurgitation (MR). If untreated, survival beyond infancy is rare. The aim of our study was to analyze the outcome in children with ALCAPA after cardiac surgery.MethodsWe retrospectively reviewed all patients who were diagnosed at our institution with ALCAPA and underwent surgical repair from 1999 to the end of 2018 (for 20 years). We followed LV dimensions, function, the progress of MV regurgitation, and the somatic growth of children after surgical repair.ResultsTwenty-nine patients underwent ALCAPA repair while 15 (52%) patients were male. The median age at surgical repair was 5.3 (IQR: 3.8-7.4) months and the mean weight was 5.5±2 kg. Surgical repair was performed in form of coronary reimplantation in 26 (90%) patients and Takeuchi repair in three (10%) patients. Intensive care unit (ICU) stay was eight (IQR: 6-17) days and hospital stay was 15 (IQR: 12-21) days. The follow-up duration was 5±3.6 years. Echocardiographic parameters started to improve by six weeks after the repair, and they normalized by one year. At the time of surgery ejection fraction (EF) was 34±17%, fractional shortening (FS) was 15±10%, and LV inner diameter in diastole (LVIDD) z score was 5.7±2.8. These parameters improved by one year after surgery to 66±7%, 34±6%, and 0±1.3, respectively. However, somatic growth started to improve six months after surgical repair. MR was moderate to severe in seven (24%) patients at the time of surgery and regressed to no more moderate nor severe MR at the last follow-up. None of the 29 patients died.ConclusionsLV systolic function and dimensions start to improve by six weeks after surgery and reach normal values by one year. MR regresses without intervention in correspondence with the regression of LV dimensional parameters. The somatic growth of children improves six months after repair.

Highlights

The anatomy of the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was first described in 1911 by Abrikossoff [1], the first comprehensive clinical description of the disease was provided in 1933 by Bland, White, and Garland
We retrospectively reviewed all patients who were diagnosed at our institution with ALCAPA and underwent surgical repair from 1999 to the end of 2018
Surgical repair was performed in form of coronary reimplantation in 26 (90%) patients and Takeuchi repair in three (10%) patients

Summary

Introduction

The anatomy of the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was first described in 1911 by Abrikossoff [1], the first comprehensive clinical description of the disease was provided in 1933 by Bland, White, and Garland. They had described in their report a threemonth-old boy with ALCAPA; since it was described as Bland-White-Garland syndrome [2,3,4]. The aim of our study was to analyze the outcome in children with ALCAPA after cardiac surgery

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