Bidirectional shunting in a coronary artery-right ventricular fistula associated with pulmonary atresia and an intact ventricular septum

A case of an anomalous arteriovenous-like communication between the right ventricle and a single right coronary artery with pulmonary atresia and an intact ventricular septum in a 6-year-old boy is presented. The dynamics of blood flow through the anomalous vessel are demonstrated for the first time in the literature by means of cineangiocardiography. Factors affecting the flow of blood through the vessel, theories of the embryologic etiology of the condition, and clinical features allowing diagnosis during life and determining therapeutic management are discussed.