Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) Syndrome

Pathophysiology and Clinical Significance

Adult survivors with ALCAPA typically have significant collateral development from the right coronary artery (RCA) to the left coronary artery (LCA), which contributes to the dilation of both vessels 1

Echocardiography may identify the dilated coronary arteries in children and young adults but has limitations in older adults 2
The American Heart Association recommends CT or magnetic resonance angiography as the initial screening method in centers with expertise in such imaging 3, 4
Coronary angiography remains valuable for delineating arterial course, branches, and assessing for atherosclerosis 2
Coronary CTA has become the "gold standard" for assessment of anomalous coronary origin and course in adults 2

The American College of Cardiology/American Heart Association guidelines recommend reconstruction of a dual coronary artery supply in patients with ALCAPA 5, 3
Surgery should be performed by surgeons with training and expertise in congenital heart disease at centers with expertise in managing anomalous coronary artery origins 5
For adult survivors of ALCAPA repair, clinical evaluation with echocardiography and noninvasive stress testing is indicated every 3 to 5 years 5, 3
Even after successful repair, residual coronary abnormalities may persist, including proximal, midvessel, and distal coronary artery obstructions 5
Patients may have residual myocardial fibrosis from preoperative ischemia, requiring ongoing surveillance 5