Anomalous right coronary artery from the pulmonary artery (ARCAPA)

Anomalous right coronary artery (RCA) from pulmonary artery is a rare congenital coronary anomaly. The prevalence is about 0.002% of general population1). Anomaly such as aortopulmonary window, tetralogy of Fallot, and septal defects has been reported in one-third of anomalous RCA originating from the pulmonary artery (ARCAPA) patients. Whereas patients with anomalous pulmonary origin of the left coronary artery (ALCAPA) usually present with signs of left ventricular ischemia and congestive heart failure in infancy, most ARCAPA patients are diagnosed during childhood with cardiac murmur, chest pain, or congestive heart failure. The diagnosis of ARCAPA is missed because of asymptomatic status2,3). However, ARCAPA may present with subclinical myocardial ischemia or is at the risk of sudden cardiac death even in early childhood.