ALCAPA repair using Takeuchi procedure in infants with cardiomyopathy and low ejection fraction, without mechanical circulatory support: case report

Abstract

Introduction: Bland-White-Garland syndrome, often referred to as the abnormal origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA), is a rare congenital cardiac disease that causes left ventricular (LV) failure and myocardial ischemia. Reimplantation of the ALCAPA into the aorta and the Takeuchi operation, which creates an intrapulmonary baffle, are two recent surgical procedures that have drastically decreased death rates to less than 20%. This study aimed to report a case of ALCAPA repair using the Takeuchi procedure in infants with cardiomyopathy and low ejection fraction without mechanical circulatory support.

Case description: A male infant 4 months old presents with choking when bottle-feeding, leading to cyanosis and shortness of breath. The patient was treated in the intensive care unit for several days, and the imaging showed dilated cardiomyopathy leading to ALCAPA. MSCT findings showed an ALCAPA with a dilated RCA diameter of 2.2 mm. Ventricular Fibrillation and cardiopulmonary resuscitation were reported during the Pediatric Intensive Care Unit (PICU) stay, then the patient decided to go for the surgical procedure. The Takeuchi procedure was done with a pericardial baffle from the left coronary going through the ascending aorta. The patient was transferred to the ICU for open chest management for four days. The patient was still hemodynamically stable afterward without mechanical circulatory support, and the LV dysfunction could still be tolerated by optimizing inotropes and after-load reduction medicines.

Conclusion: ALCAPA leads to myocardial ischemia and left ventricular dysfunction, posing significant risks, especially in infancy. Timely surgical intervention is essential to improve outcomes, with recent advancements in surgical techniques showing promising results in reducing mortality rates in case of severe LV dysfunction without mechanical circulatory support (ECMO or LVAD).