BACKGROUND: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly with an incidence of 0.002%.

CASE REPORT: A 58-year-old African American female with a history of diabetes mellitus, hyperlipidemia, and hypertension was evaluated for shortness of breath and chest heaviness. On physical examination, she was found to be morbidly obese. Her blood pressure was 160/90 mmHg. There were no carotid bruits or jugular venous distension. Cardiac auscultation showed distant heart sounds with no audible murmurs. Lower extremity examination showed +1 edema with weak pedal pulses. ECG showed non-specific ST segment and T-wave changes. Echocardiogram demonstrated dysfunction grade I with preserved ejection fraction. An adenosine nuclear study showed an area of reversible ischemia of the inferior wall. Selective left coronary angiography showed the left coronary artery (LCA) originating from the left sinus of Valsalva. From the LCA, the left anterior descending and the left circumflex coronary arteries arose in a typical course. The right coronary artery (RCA) was visualized in a retrograde fashion via collaterals originating from the left coronary system and it drained into the pulmonary artery. On aortic root angiography, the origin of the RCA could not be determined. The patient’s surgical risk was deemed unacceptably high and she was not considered a surgical candidate. Her symptoms were controlled conservatively.

CONCLUSIONS: By reporting this case, we shed some light on a rare congenital anomaly involving the coronary arteries. Variable presentations have been described for ARCAPA, however, many patients remain asymptomatic. Diagnosis can be confirmed by coronary angiography. Surgical correction is the definitive treatment.

Keywords: Coronary Vessel Anomalies, Coronary Vessels, Pulmonary Artery