Analysis of Perioperative and Long-Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy

Nadine Straka; Kimberlee Gauvreau; Yisong Huang; Elizabeth DeWitt; Catherine Allan; Ravi Thiagarajan; Meena Nathan

doi:10.1007/s00246-023-03344-1

Analysis of Perioperative and Long-Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy

Pediatr Cardiol. 2023 Dec 15. doi: 10.1007/s00246-023-03344-1. Online ahead of print.

Authors

Nadine Straka^{1

2}, Kimberlee Gauvreau³, Yisong Huang³, Elizabeth DeWitt³, Catherine Allan³, Ravi Thiagarajan^#³, Meena Nathan^#⁴

Affiliations

¹ Department of Pain, Critical Care, and Anesthesia, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA. nadine.straka@childrens.harvard.edu.
² Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA. nadine.straka@childrens.harvard.edu.
³ Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA.
⁴ Department of Cardiac Surgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02115, USA.

^# Contributed equally.

PMID: 38099949
DOI: 10.1007/s00246-023-03344-1

Abstract

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. A retrospective chart review of patients who underwent ALCAPA repair between 1996 and 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. 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. 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.

Keywords: ALCAPA; Anomalous left coronary artery from the pulmonary artery; Late presentation; Left ventricular dysfunction; Mitral regurgitation; Outcomes.