To determine the impact of the Fontan operation on exercise tolerance and on the cardiorespiratory response to exercise, we compared the results of graded exercise to maximal effort of 81 patients with tricuspid atresia or single functional ventricle studied preoperatively with those of 29 patients studied postoperatively. Postoperatively, the values for total work performed, duration of exercise and maximal oxygen uptake increased significantly. Regardless of operative status, the maximal heart rate during exercise was reduced. The cardiac output and stroke volume response to exercise were subnormal after operation. Systemic arterial blood oxygen saturation was reduced markedly preoperatively both at rest and during exercise; postoperatively, it was significantly greater than the preoperative value but it remained slightly abnormal. The ventilatory response to exercise (respiratory rate, minute ventilation and ventilatory equivalent for oxygen) decreased toward normal after operation. Exercise tolerance and the cardiorespiratory responses to exercise improve after the Fontan operation. Formal exercise testing is essential to quantitate the degree of improvement.