Anomalous Right Coronary Artery from the Pulmonary Artery: Diagnosis and Management

Pathophysiology and Clinical Presentation

Adult survivors of ARCAPA typically develop extensive collateral networks from the left coronary artery to the anomalous right coronary artery, contributing to dilation of both vessels, as noted by Praxis Medical Insights 1

The American Heart Association recommends CT or magnetic resonance angiography as the initial screening method in centers with expertise in such imaging for assessment of anomalous coronary origin and course in adults 1
Coronary CT angiography has become the gold standard for assessment of anomalous coronary origin and course in adults, according to Praxis Medical Insights 1
Echocardiography can identify dilated coronary arteries in children and young adults but has limitations in older adults, as stated by Praxis Medical Insights 1
Invasive coronary angiography remains the definitive diagnostic test and is valuable for delineating arterial course, branches, and assessing for atherosclerosis, as noted by Praxis Medical Insights 1

The American College of Cardiology/American Heart Association guidelines recommend reconstruction of a dual coronary artery supply in patients with ARCAPA, with surgery performed by surgeons with training and expertise in congenital heart disease at centers with expertise in managing anomalous coronary artery origins 2, 1

For adult survivors of ARCAPA repair, clinical evaluation with echocardiography and noninvasive stress testing is indicated every 3 to 5 years, as recommended by the American College of Cardiology/American Heart Association and Praxis Medical Insights 2, 1
Even after successful repair, residual coronary abnormalities may persist, including proximal, midvessel, and distal coronary artery obstructions, as noted by Praxis Medical Insights 1
Patients may have residual myocardial fibrosis from preoperative ischemia, requiring ongoing surveillance, as stated by Praxis Medical Insights 1

Surgery is recommended for ARCAPA to establish a dual coronary artery supply, performed by surgeons with congenital heart disease expertise at specialized centers, as recommended by the American College of Cardiology 3, 4, 5, 6, 7

Consider preoperative myocardial fibrosis from chronic ischemia when planning revascularization strategy, according to the American Heart Association 8

Implement a multimodal approach with transfusion algorithms and point-of-care testing to limit perioperative transfusions, as suggested by the American College of Cardiology 9
Lysine analogues, such as tranexamic acid or aminocaproic acid, should be administered intraoperatively to reduce blood loss and transfusion requirements, as recommended by the American College of Cardiology 9

Clopidogrel and ticagrelor should be withheld for at least 5 days before surgery, according to the American College of Cardiology 9
Prasugrel should be withheld for at least 7 days before surgery, as recommended by the American College of Cardiology 9
Tirofiban or eptifibatide should be discontinued 2-4 hours before surgery, and abciximab at least 12 hours before surgery, according to the American College of Cardiology 9