Repair of anomalous left coronary artery from pulmonary artery (ALCAPA) beyond infancy

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiac Surgery

Background:

• Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect.
• ALCAPA typically presents in infancy, often with severe symptoms like heart failure.
• Late presentation in older children and adults is uncommon but documented.

Purpose of the Study:

• To evaluate surgical outcomes for patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) presenting beyond infancy.
• To assess the feasibility and long-term results of corrective surgery in a cohort of older ALCAPA patients.

Main Methods:

• Retrospective review of five patients (3-56 years) who underwent corrective surgery for ALCAPA between 2002 and 2012.
• Surgical techniques included direct aortic implantation (coronary-button transfer) and coronary artery bypass with ostial obliteration.
• Concomitant procedures included mitral valve repair and ventricular septal defect closure.

Main Results:

• Four out of five patients survived surgery with good long-term outcomes.
• Survivors achieved New York Heart Association (NYHA) class I status with preserved left ventricular ejection fraction (45-60%).
• Postoperative mitral insufficiency ranged from none to moderate.

Conclusions:

• Selected patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) can survive beyond infancy.
• Surgical correction can establish dual coronary circulation with satisfactory functional results.
• Early diagnosis and intervention remain crucial, but surgical options exist for older, naturally selected patients.