Analysis of perioperative and long-term outcomes among presentations of anomalous left coronary artery from the pulmonary artery diagnosed beyond infancy versus during infancy

Abstract

Abstract Background Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) typically presents in infancy; however, there are cases of patients who survive the infant period and present later in life. We aimed to characterize patients with late ALCAPA diagnoses and to assess perioperative and functional outcomes. Methods A retrospective chart review of patients who underwent ALCAPA repair between 1996 to 2020 at Boston Children’s Hospital was performed. This cohort was divided into early ALCAPA (<1 year) and late ALCAPA (≥ 1 year) groups. Perioperative data were collected. Longitudinal functional assessments were made by echocardiography, exercise stress test, and cardiac magnetic resonance imaging. Results The median age of the late ALCAPA group was 7.6 years with 25% (6/24) of patients over 18 years. The late ALCAPA group was more likely to present as an incidental finding (63%) and required less preoperative intervention compared to the early group. On preoperative echocardiogram, the late ALCAPA group had less moderate or severe mitral regurgitation (16.7% vs 62%, p<0.001) or left ventricular dysfunction (16.7% vs 89%, p <0.001) compared to the early group. Reoperation was uncommon and both groups demonstrated almost complete resolution of mitral regurgitation and left ventricular dysfunction over time. Conclusion There are important differences between late and early ALCAPA subtypes. Revascularization results in excellent outcomes in both early and late groups but long-term surveillance of ALCAPA patients is warranted as they may have functional deficits after repair.