Surgical Reimplantation of the Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery (ARCAPA)

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary artery anomaly, accounting for less than 0.002 percent of all congenital heart defects. Unlike its more frequently encountered counterpart—anomalous left coronary artery from the pulmonary artery (ALCAPA)—ARCAPA often remains undetected due to its frequently asymptomatic presentation, particularly in early life. However, it can lead to significant clinical consequences, including myocardial ischemia, arrhythmias, or sudden cardiac death, especially under conditions of increased myocardial demand. The condition is characterized by the right coronary artery arising from the main pulmonary artery, resulting in a left-to-right shunt via collateral circulation from the left coronary system. This coronary steal phenomenon can compromise myocardial perfusion. Diagnosis is typically made using imaging modalities such as echocardiography, coronary angiography, or cardiac computed tomography (CT)/magnetic resonance imaging (MRI). Surgical correction, most commonly via reimplantation of the right coronary artery (RCA) into the aorta or ligation with bypass grafting, is recommended to restore dual coronary circulation and prevent long-term complications.  

The authors present a case of ARCAPA in a six-month-old child, which was diagnosed by echocardiography and confirmed by multislice computed tomography (MSCT). The authors’  preferred technique was reimplantation of the RCA to the aorta and reconstruction of the pulmonary artery by autologous pericardium. 

References

1. Al-Dairy A, Rezaei Y, Pouraliakbar H, Mahdavi M, Bayati P, Gholampour-Dehaki M. Surgical repair for anomalous origin of the right coronary artery from the pulmonary artery. Korean Circ J 2017; 47: 144–147
2. Guenther TM, Sherazee EA, Wisneski AD, Gustafson JD, Wozniak CJ, Raff G. Anomalous origin of the right coronary artery from the pulmonary artery: a systematic review. Ann Thorac Surg. 2020;110(3):1063-1071.
3. Guenther TM, Sherazee EA, Wisneski AD, Gustafson JD, Wozniak CJ, Raff G. Anomalous origin of the right coronary artery from the pulmonary artery: a systematic review. Ann Thorac Surg. 2020;110(3):1063-1071.

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