Impact of Adjustable Pulmonary Artery Band on Outcomes of Anomalous Left Coronary Artery From the Pulmonary Artery (ALCAPA) Repair

Objective: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly resulting in myocardial ischemia, global left ventricular (LV) dysfunction, and mitral regurgitation (MR). Surgical placement of a pulmonary artery band (PAB) at ALCAPA repair may assist with recovery of patients with severe LV dysfunction. We assessed the outcomes of patients with ALCAPA and severe dysfunction treated with, and without a PAB. Methods: We performed a retrospective analysis of cases presented to our center between January 2007 and December 2023. Baseline and follow-up data were collected from records. Ventricular function was assessed by standardized transthoracic echocardiogram. Results: Twenty-six patients underwent ALCAPA repair at a median age of 4 months (range 19 days-8 years). Surgery was performed by direct coronary reimplantation in all patients. Beginning in 2017, we have placedan adjustable PAB in 5 patients with severe LV dysfunction (<25% ejection fraction [EF]). No patients required mitral valve repair at the time of ALCAPA surgery. Two patients with EF < 25% from the non-PAB group required support with extracorporeal membrane oxygenation (ECMO). In patients with EF < 25% who received PAB, none required ECMO or transplant. Recovery of function was near normal in the patients with EF < 25% plus a PA band, but did not reach normal in those with no PA band. Conclusion: There is marked improvement of MR grade and LV function, and no mortality during a 10-year period in the group with EF > 25%. Patients with EF < 25% treated with a PAB demonstrated an improved trajectory toward EF improvement, achieving normal function earlier than those without PA band. Temporary adjustable PAB may be beneficial in ALCAPA patients with severe dysfunction.