In a series of 74 heart transplant recipients undergoing annual coronary angiography, a coronary artery to right ventricle fistula was observed in 4 patients, an incidence rate of 5.4%, which is much higher than the expected incidence of congenital coronary artery fistula (0.1% to 0.2%). A traumatic origin of the fistulas is unlikely because none of the heart donors had evidence of chest trauma. An endomyocardial biopsy-related etiology of the fistulas is postulated. All fistulas were located in the biopsy sampling area. Patients with a fistula underwent more biopsies before the diagnosis compared with patients without a fistula (20 +/- 11 versus 14 +/- 6, p = 0.05). At least one large arteriole (diameter greater than 0.16 mm) was found on pathologic examination of the biopsy specimens from each of the patients with a fistula (100%) but in only 2 (16.7%) (p less than 0.01) of 12 randomly selected patients without a fistula. The size of the fistula appears to be hemodynamically insignificant in all four patients, judging from angiographic size, normal intracardiac pressures and normal cardiac output values at rest. The diagnosis of a coronary artery to right ventricle fistula is possible and should be entertained at the time of coronary angiography of heart transplant recipients. The clinical significance of the finding is unclear. As long as endomyocardial biopsy remains the diagnostic method of identifying tissue rejection, prevention of the described complication is unlikely.