Congenital coronary artery fistula: report of five patients, diagnostic problems and principles of management.

The detection of a coronary artery fistula (CAF) is usually by chance and, because of the to-and-fro characters of the murmur, a misdiagnosis of a patent ductus arteriosus is frequently made. Correct diagnosis can only be reached after hemodynamic investigation completed by angiography. This lesion is often accompanied by few or no symptoms in infancy, but may become symptomatic with aging even if the shunt is small: complications such as endocarditis, rhythm disturbances, aneurysmatic dilation and rupture have been reported. Five cases of CAF have been studied in our institution: 3 of them have been operated upon in extracorporeal circulation with good results. Principles of management are controversial: many authors advocate delay of treatment until symptoms appear: in our opinion, based on the natural history of the disease and on our experience, closure of the fistula in pediatric age, even in asymptomatic patients, is the treatment of choice.