Normal children achieve the same increase of oxygen uptake (VO2) in response to exercise even though resting and submaximal exercise heart rates vary greatly as a function of age, body size and physical conditioning. To determine whether the VO2 response to exercise is altered when heart rate is significantly reduced by heart disease, we compared 78 children who achieved a peak exercise heart rate of less than or equal to 150 beats/min to 201 controls of similar body size and normal peak exercise heart rates of greater than or equal to 180 beats/min. All performed incremental (16.4 Watts/min) maximal cycle exercise. Separate analysis of males and females included heart rate, power (kg-m/min, Watts/kg), VO2 (ml/min, ml/min per kg), O2 pulse (VO2/heart beat), VE (l/min) and R (VCO2/VO2) at rest and during the 1st, 4th and last minute of exercise. Patients with low peak exercise heart rates had also lower resting submaximal exercise heart rates than controls. VO2 at comparable exercise levels did not differ from controls and consequently O2 pulse was greater in the patients than controls at rest and at all levels of exercise. A consistent gender difference was only found in controls where males achieved a higher VO2 and lower heart rates at comparable levels of exercise. The data show a normal exercise VO2 despite significantly lower heart rates. These findings cannot be explained by an increased arteriovenous difference alone and suggest that the patients retained the ability to effectively modulate stroke volume.