Severely dilated right coronary artery in an infant with pulmonary atresia with intact ventricular septum (PA/IVS)

A severely dilated right coronary artery was observed in a 1-week-old infant with PA/IVS undergoing modified Blalock-Thomas-Taussig shunt. In patients with PA/IVS and a small tricuspid valve, right ventricle-to-coronary fistulae may develop. If the normal origins of these coronary arteries are stenotic or atretic, the heart is dependent on right ventricular coronary filling. This patient had a tortuous and severely dilated right coronary artery, which was noted to have ostial stenosis, and an intact left coronary artery.