A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum

A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant bo th coronary arteries lacked their normal proximal connection with the aorta , perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. Thi s patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.