BACKGROUND: Cameral coronary artery fistulae (C-CAFs) are common after heart transplantation (HTx) and typically drain into the right ventricle. The development of CAFs to non-cameral structures after HTx has not been systematically investigated. We studied the incidence, anatomic distribution, and natural history of non-cameral CAFs (NC-CAFs) in a multicenter pediatric population. METHODS: Medical records from pediatric HTx patients at 2 centers from January 1, 1999, to August 31, 2009 were reviewed. A classification system for CAF size was developed, and serial angiograms were evaluated for CAF presence, size, and anatomy. Risk factors and outcomes were determined. RESULTS: Identified were 100 patients with a median age at HTx of 8.7 years. Median follow-up was 4.2 years. NC-CAFs occurred in 52 patients, C-CAFs in 20, with both types noted in 11. NC-CAFs originated from coronary arteries and drained predominantly into ipsilateral recipient pulmonary vasculature. Multiple NC-CAFs occurred in 19 patients (19%) for a total of 77 fistulae in 52 patients. Fistulae were classified as small (56%), moderate (36%), and large (8%). NC-CAFs were present at the first post-HTx angiogram in 95% of cases (median 1 year after HTx). Longer donor ischemic time was associated with the development (p = 0.043) and size (p = 0.05) of NC-CAFs. NC-CAFs were not associated with rejection, death, re-transplantation, or coronary revascularization, and none required intervention. CONCLUSIONS: Fistulae from donor coronary arteries to recipient pulmonary vasculature develop frequently and early after pediatric HTx. These correlate with graft ischemic time, but are not associated with early graft loss or death.