The incidence, angiographic characteristics, and natural history of coronary artery fistulas in patients undergoing diagnostic cardiac catheterization have not been well defined. Of 33,600 patients who had diagnostic cardiac catheterization, 34 (0.1%) had coronary artery fistula. Nineteen fistulas originated from the right, 11 from the left anterior descending, and 4 from the circumflex coronary arteries, respectively. The mean ratio of pulmonary to systemic flow was 1.19 +/- 0.33. Only one patient with coexistent atrial septal defect had a pulmonic to systemic flow ratio > 1.5. Right and left heart pressures, with the exception of three patients in whom left ventricular end-diastolic pressures was > 12 mm Hg, were within normal limits. During a mean follow-up period of 6.3 years (range 2-14 years), there were no complications related to coronary artery fistula. It was concluded that the incidence of coronary artery fistulas detected during diagnostic coronary angiography is very low. Coronary artery fistulas originate predominantly from the right coronary artery and are not associated with hemodynamic abnormalities or other congenital heart diseases. The prognosis of coronary artery fistulas in adults is good.