Ectatic-Fistulous Coronary Artery Lesion: A Newly Proposed Nomenclature

Congenital Coronary Artery Fistulae (CAF) originating from Right Coronary Artery (RCA) and draining into Coronary Sinus (CS) demonstrate high gradients and high flows, which result in ectasia of the entire artery. The anatomic margin between the ectatic RCA and the fistula are not clear and most cardiologists and cardiac surgeons tend to misname the entire vessel as "fistula". We presented a 60-year-old female with multiple CAF draining into CS. The fistula originating from the RCA was huge and caused severe ectasia of the entire artery. The patient had progressive breathlessness due to high left to right shunt. Both fistulae were ligated under cardiopulmonary bypass. Our introduced term "ectatic-fistulous lesion" comes to address the confusion with nomenclature. It not only offers the theoretical advantage of precise description of the above complex pathology, but also allows the cardiac team to identify the anatomical margins between the ectasia and the fistula and, thus, to proceed to closure of the coronary fistula with safety.