A Rare Combination—Aortopulmonary Window With Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery

A 9-day-old neonate weighing 2.2 kg, who had passed the neonatal screen for critical congenital heart disease, was re-admitted with heart failure symptoms, including lethargy and failure to thrive. A murmur was heard, and a transthoracic echocardiogram confirmed a large aortopulmonary window (APW) and the origin of the right coronary artery (RCA) from the main pulmonary artery (ARCAPA). 

Through standard median sternotomy, cardiopulmonary bypass was initiated. After achieving cardioplegic arrest, the APW was completely divided. The RCA was found to be arising from the APW and was relocated back to the ascending aorta during the division of the APW. To take enough button on the RCA and safely divide the APW, the facing commissure of the pulmonary valve was detached. A decellularized pulmonary homograft patch was used to reconstruct both the aortic and pulmonary artery walls. The detached commissure was reattached to the pulmonary artery wall patch. 

The patient was weaned off cardiopulmonary bypass without difficulty, and the chest was closed 24 hours later. The remaining postoperative course was uneventful; the patient was extubated on the second postoperative day and was discharged a week later. The patient continued to do well during follow-up, and an echocardiogram showed excellent biventricular function, good flow in the RCA, and competent aortic and pulmonary valves. 

References

1. Asano M, Ukai T, Nomura N, Mishima A. Anatomical repair of aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery. J Card Surg. 2013 Nov;28(6):767-9
2. Greenway SC, Bradley TJ, Caldarone CA, Silverman NH, Hanley FL, Smallhorn JF. Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies. Eur J Echocardiogr. 2006 Oct;7(5):379-82

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