Clinical and angiographic analysis of congenital coronary artery fistulae in adulthood. Is there any new trend?

Fifteen (2.1%) patients were diagnosed as having congenital coronary artery fistula (CAF) in a consecutive series of 704 adult patients undergoing selective coronary arteriography; the incidence was astonishingly higher than previous observations. The presentation of clinical symptoms and the electrocardiographic changes at rest and/or after exercise testing probably attributable to the CAF were observed unexpectedly often in spite of the fact that the magnitude of the shunt seemed not to be significant. With respect to the anatomy of the CAF, the incidence of origination from plural coronary arteries and that of fistulation into the left ventricle were also unexpectedly high. These observations presented a striking contrast to those of previous reports, but we were unable to determine the reason. In four cases, the CAF was ligated either electively or concurrently with mitral valve surgery, and the results were satisfactory. Taking these circumstances into consideration, we should not minimize the impact of a CAF with a seemingly small shunt.