This study evaluates right ventricular (RV) and pulmonary function during exercise in adults with congenital heart disease (CHD). Thirty-one patients with CHD involving the right side of the heart underwent symptom-limited bicycle exercise testing with simultaneous expired gas analysis and measurement of RV ejection fraction (EF). Twenty-one age-matched normal controls underwent the identical exercise protocol. Maximal oxygen consumption was lower in the CHD than in normal controls (19.5 +/- 6.4 vs 30.5 +/- 0.8 ml/kg/min, p = 0.0001 patients vs controls). Both heart rate (156 +/- 25 vs 171 +/- 13 beats/min, p = 0.01) and oxygen pulse (9.3 +/- 3.7 vs 12.3 +/- 3.7 ml/beat, p = 0.01), an indirect measure of stroke volume, were found to be lower in the CHD group at peak exercise. Pulmonary dysfunction was evidenced in the CHD group by decreased forced expiratory volume, forced vital capacity and maximum voluntary ventilation, and by a higher ventilation/expired carbon dioxide ratio at peak exercise (37.2 +/- 6.9 vs 33.0 +/- 5.4, p = 0.02), suggesting an increase in dead space ventilation. Maximal oxygen consumption was lower in patients whose RVEF decreased with exercise (17.6 +/- 5.4 vs 22.8 +/- 6.4 ml/kg/min, p = 0.03 "decrease RVEF" group vs "increase RVEF" group). Maximal oxygen consumption correlated with the change in RVEF only in the group whose RVEF decreased with exercise (r = 0.5, p = 0.03). In the group that had increased RVEF with exercise, maximal oxygen consumption correlated with forced expiratory volume (r = 0.7, p = 0.02). Thus, adults with CHD have a reduced functional capacity compared with normal controls. This phenomenon appears to be associated with both RV and pulmonary abnormalities.