This study aimed to (a) examine the influence of type I diabetes on the cardiopulmonary exercise response in trained subjects and (b) determine whether glycemic control affects these responses. The cardiopulmonary responses to maximal incremental cycle ergometry were compared in 12 Ironman triathletes with type I diabetes and 10 age- and sex-matched control subjects without diabetes. Athletes with type I diabetes were then stratified into low- (glycosylated hemoglobin (HbA1c) < 7%, n = 5) and high-HbA1c (HbA1c > 7%, n = 7) groups for comparison. Cardiac output, stroke volume, arterial blood pressure, and calculated systemic vascular resistance along with airway function were measured at rest and during steady-state exercise. During peak exercise HR, stroke volume and cardiac output were not different between the groups with and without diabetes; however, forced expiratory flow at 50% of the forced vital capacity was lower in subjects with diabetes (P < 0.05). Within the group with diabetes, HbA1c was lower in the low-HbA1c versus high-HbA1c group (6.5 +/- 0.3 vs 7.8 +/- 0.4, respectively; P < 0.05), but training volume was not different. At rest, the low-HbA1c group had greater cardiac output and lower systemic vascular resistance than the high-HbA1c group, and all pulmonary function measurements were greater in the low-HbA1c group (P < 0.05). During peak exercise, the VO2, workload, HR, stroke volume, and cardiac output were greater in the low-HbA1c versus the high-HbA1c group (P < 0.05). In addition, all indices of pulmonary function were higher in the low-HbA1c group (P < 0.05). Finally, within the subjects with diabetes, there was a weak inverse correlation between HbA1c and exercise training volume (r2 = -0.352) and stroke volume (r2 = -0.339). These data suggest that highly trained individuals with type I diabetes can achieve the same cardiopulmonary exercise responses as trained subjects without diabetes, but these responses are reduced by poor glycemic control.