Surgical Repair of an Anomalous Left Coronary Artery From the Right Pulmonary Artery

Surgical repair of anomalous left coronary artery from the right pulmonary artery is indicated once diagnosed, especially in infants presenting with severe ventricular dysfunction, with or without mitral regurgitation or myocardial ischemia.

Case Presentation

A 4-month-old male infant (6.3 kg) presented with heart failure symptoms.

Preoperative transthoracic echocardiography showed:

• Hyperechoic mitral papillary muscle
• Dilated left atrium and left ventricle
• Severe left ventricular (LV) dysfunction
• Retrograde flow in the circumflex coronary artery
• Mild mitral and tricuspid regurgitation

Chest computed tomography (CT) was inconclusive but highly suspicious for anomalous origin of the left coronary artery from the pulmonary artery. Coronary angiography confirmed the anomalous left coronary artery originating from the right pulmonary artery, showing a dilated right coronary artery (RCA) supplying collateral flow to the left coronary artery (LCA), which was filled retrogradely and emptied into the pulmonary artery.

Operative Steps

After median sternotomy and harvesting the pericardial patch, the individual pulmonary arteries (PA) were dissected to the branching point and looped before initiation of cardiopulmonary bypass (CPB) to control potential coronary steal. After establishing CPB, the pulmonary arteries were snared, followed by application of the aortic cross-clamp and infusion of cardioplegia into the aortic root.

The ascending aorta was transected slightly higher than usual, considering the possibility that the anomalous coronary artery originated from the right pulmonary artery and to minimize kinking after anastomosis to the aorta. After transection, the surgeons confirmed the single right coronary artery (RCA) opening in the corresponding sinus, while the other two sinuses were empty. The main pulmonary artery was opened just proximal to the confluence, and the anomalous LCA arising from the right pulmonary artery (RPA) was identified. The LCA button was harvested with a generous cuff of PA tissue.

The coronary button was implanted into the aorta by creating a posterior slit to avoid tension or kinking. The PA defect was reconstructed using an autologous pericardial patch, which should be redundant and should not cause any pressure effect on the reimplanted coronary artery. Before aortic closure, cardioplegia was infused into the LCA ostium to confirm the absence of collateral back-bleeding, and then the aortotomy was closed. The cross-clamp was removed, and the patient was weaned from CPB with satisfactory hemodynamics.

Preference Card

• Standard pediatric cardiac instrument set
• Polypropylene coronary sutures
• Autologous pericardial patch for PA reconstruction
• Cardioplegia delivery cannula for coronary ostial testing

Tips and Pitfalls

• Loop the branch pulmonary arteries before the initiation of CPB to control coronary steal.
• Harvest a generous coronary button to prevent tension during reimplantation.
• Ensure a sufficiently high aortotomy for optimal coronary alignment.
• Carefully select the reimplantation site on the ascending aorta to avoid kinking.
• Perform ostial cardioplegia testing to confirm coronary patency and absence of collateral back-bleeding before aortic closure.

Outcome

The postoperative course was uneventful. The patient was extubated on postoperative day three to high-flow nasal cannula (HFNC) for 24 hours. Inotropes were weaned on postoperative day four, and the patient was discharged with stable hemodynamics.

Key Learning Point

Direct coronary transfer restores a two-coronary artery system and remains the preferred surgical strategy for anomalous left coronary artery from the right pulmonary artery.

References

1. Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Kirklin/Barratt-Boyes Cardiac Surgery. 4th ed. Elsevier; 2013.
2. Agrawal V, Vaidhya N, Patel M, Mishra A, Patel D. Intramural Aortic Course Should Always Be Considered for Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery. World J Pediatr Congenit Heart Surg. 2019;10(4):508-512. doi:10.1177/2150135118799645
3. Mishra A. Surgical management of anomalous origin of coronary artery from pulmonary artery. Indian J Thorac Cardiovasc Surg. 2021;37(Suppl 1):131-143. doi:10.1007/s12055-021-01147-8

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