Anomalous Origin of the Right Coronary Artery from Pulmonary Artery With Ostial Stenosis
A 4-year-old girl was referred for evaluation of a presumed coronary artery fistula, incidentally detected by echocardiography in infancy. She was followed up clinically without having any symptoms. An echocardiogram showed continuous flow in the proximal main pulmonary artery (Movie I of the online-only Data Supplement). The left coronary artery was prominent and dilated. Probable collateral vessels were seen in the ventricular septum and right ventricle free wall. There was no regional wall motion abnormality, and ventricular function was normal. The ECG was normal, without any evidence of ischemia.
Cardiac catheterization was arranged for further evaluation and possible coil embolization of the fistula at the same time. A selective left coronary angiogram (Movie II of the online-only Data Supplement) demonstrated a prominent left main coronary artery, a normal left anterior descending artery, and normal distribution of the circumflex artery. The left main coronary artery arose from the appropriate sinus without aneurysm or stenosis. After a slight delay, the posterior descending and right coronary artery (RCA) opacified in a retrograde fashion through collaterals of the left coronary system. The right coronary artery drained into the main pulmonary artery. The right coronary ostium was severely stenotic (Figure). The patient subsequently underwent surgical reimplantation of the right coronary to aortic sinus.
Anomalous right coronary artery from pulmonary artery is a very rare congenital coronary artery anomaly.1,2 In about 50% of cases, anomalous right coronary artery from pulmonary artery is associated with other congenital heart defects. Whereas patients with anomalous pulmonary origin of the left coronary artery usually present in infancy with signs of left ventricular ischemia and congestive heart failure, those with anomalous origin of the RCA may remain asymptomatic until adulthood.3 Presentations may include exertional chest pain syncope, sudden cardiac death, or findings of ischemic cardiomyopathy. Although the diagnosis is usually made echocardiographically, coronary angiography, if indicated, is definitive. Even in asymptomatic patients, surgical correction is mandatory. Given this patient’s ostial stenosis at the origin of the right coronary artery in the pulmonary artery, the usual degree of coronary runoff into the low-pressure pulmonary artery was limited. Furthermore, collateralization between the left and right coronary vascular beds helped maintain adequate myocardial perfusion, and at presentation our patient was essentially asymptomatic, with no manifest evidence of coronary ischemia.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/120/23/e282/DC1.