Correcting a Rare Congenital Anomaly in an Adult: A Case Report
Anomalous origin of the right coronary artery (RCA) from the pulmonary artery is an extremely rare condition with an uncertain natural history. Here, the authors present an otherwise healthy active duty service member who was found to have an anomalous RCA arising from the pulmonary artery as an incidental discovery during a workup for possible pulmonary embolus.
The authors describe the preoperative workup, which showed dilation of the coronary arteries and evidence of ischemia. The operative technique for restoration of a two-vessel coronary system is described.
There are several case reports in the literature of patients with sudden cardiac death from this condition. This patient underwent an uncomplicated transposition of the RCA from the pulmonary artery to the aorta in an end-to-side fashion with a patch repair of the pulmonary artery. Postoperative imaging demonstrated reduction in the coronary dilation and resolution of ischemia.
Surgical repair, which can include simple ligation of RCA, ligation of RCA with bypass, or reimplantation of the RCA into the aorta, should be pursued regardless of symptoms, with the goal of eliminating the left-to-right shunt and establishing dual coronary flow. This case report demonstrates the successful technique of reimplanting the RCA with patch repair of the pulmonary artery, with care taken to minimize cross-clamp time.
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