What is the recommended intraoperative management of Anomalous Right Coronary Artery arising from the Pulmonary Artery (ARCAPA)?

Intraoperative Management of ARCAPA

Primary Surgical Approach

Surgery is recommended for ARCAPA to establish a dual coronary artery supply, performed by surgeons with congenital heart disease expertise at specialized centers. 1, 2

Surgical Technique Selection

The surgical goal is reconstruction of a dual coronary artery system through one of the following approaches:

Preferred Technique: Reimplantation

• Direct reimplantation of the anomalous RCA into the ascending aorta is the superior method for restoring physiological myocardial blood supply 3, 2
• This technique preserves native coronary anatomy and provides optimal long-term perfusion 3

Alternative Technique: Bypass Grafting with Ligation

• Ligation of the anomalous RCA combined with coronary artery bypass grafting (typically saphenous vein graft to the posterior descending artery) is a less traumatic alternative when reimplantation is technically challenging 4
• This approach is recognized as appropriate when the RCA wall is thin and fragile due to chronic low-pressure perfusion 3
• Internal mammary artery grafts should be reserved for left-sided targets if concurrent left coronary disease exists 4

Critical Intraoperative Considerations

Anatomic Assessment

• The anomalous RCA functions as a low-pressure, thin-walled vessel serving as a retrograde venous conduit from the left coronary circulation into the pulmonary artery 3
• Extensive collateral networks from the left coronary artery to the RCA are typically present, causing dilation of both vessels 2, 5
• The left-to-right shunt through the anomalous connection results in elevated pulmonary artery oxygen saturation and high cardiac output 3

Hemodynamic Management

• Maintain adequate systemic perfusion pressure to preserve collateral flow to the RCA territory during manipulation 3
• Monitor for myocardial ischemia in the RCA distribution, particularly the inferior and inferolateral walls 4, 6
• Be prepared for potential hemodynamic instability when ligating or manipulating the anomalous vessel 3

Concurrent Pathology

• Assess for and address concomitant valvular disease, particularly mitral regurgitation, which may coexist with ARCAPA 6
• Evaluate for atherosclerotic disease in the left coronary system, as patients may have concurrent obstructive coronary disease requiring additional bypass grafting 4
• Consider preoperative myocardial fibrosis from chronic ischemia when planning revascularization strategy 1, 2, 5

Blood Conservation Strategy

Implement a multimodal approach with transfusion algorithms and point-of-care testing to limit perioperative transfusions 1

• Lysine analogues (tranexamic acid or aminocaproic acid) should be administered intraoperatively to reduce blood loss and transfusion requirements 1
• Consider off-pump techniques if feasible to reduce bleeding, though this is rarely applicable for ARCAPA repair 1

Antiplatelet Management

If the patient is on antiplatelet therapy preoperatively:

• Clopidogrel and ticagrelor should be withheld for at least 5 days before surgery 1
• Prasugrel should be withheld for at least 7 days before surgery 1
• Tirofiban or eptifibatide should be discontinued 2-4 hours before surgery, and abciximab at least 12 hours before surgery 1

Common Pitfalls to Avoid

• Do not assume asymptomatic presentation indicates low surgical urgency—sudden cardiac death risk persists even without symptoms 4, 3, 6
• Avoid simple ligation without revascularization, as this fails to restore adequate myocardial perfusion 4
• Do not underestimate the fragility of the anomalous RCA wall during surgical manipulation 3
• Recognize that normal preoperative stress testing does not eliminate the need for surgical correction 3, 6