Multiple proximal coronaro-pulmonary fistulae. Review of the literature apropos of a new case

The authors report a new case of multiple proximal coronaro-pulmonary fistula between right coronary arteries, anterior interventricular artery and the trunk of the pulmonary artery, in a 64 year-old female patient with chest pain and a continuous murmur located in the third left intercostal space. The coronary steal is demonstrated by a myocardial scintigraphy during stress with return to normal after surgical ligation. A review of the literature enabled to find 33 cases of this major congenital anomaly of the coronary arteries, defined as an abnormal communication between at least two main coronary vessels and the trunk of the pulmonary artery. This results in a left-right shunt, usually minor without any repercussions on the right cavities and pulmonary pressures. The entire clinical, electrocardiographic, radiological, sonographic, scintigraphic, haemodynamic and angiographic picture is reported for these 33 cases. A physiopathological discussion is proposed. The course of this disease is usually favorable (only one case of myocardial infarction was published, without cardiac failure. Osler's endocarditis or sudden death); this seems to authorize simple monitoring as a logical therapeutic approach except when a myocardial ischemia secondary to coronary steal is demonstrated, imposing a surgical correction.