The physiologic role of the right ventricle has long been a subject of interest to physiologists. The Fontan operation provides a human model for studying the circulation in series devoid of a subpulmonic right ventricle. The hemodynamic response to isotonic exercise in this setting has been established, and differs appreciably from normal. However, the physiologic response to an increase in heart rate (atrial pacing) as an isolated variable has not been examined and compared to atrial pacing in hearts with two concordant subarterial ventricles. Accordingly, we compared the supine bicycle exercise response to rate-equivalent right atrial pacing in nine patients after atriopulmonary anastomoses (the Fontan operation) for single ventricle or tricuspid atresia. Cardiac index increased 77% with exercise (rest 2.6 L/min/m2; exercise 4.6 L/min/m2) but decreased 12% with atrial pacing (rest 2.5 L/min/m2; pacing 2.2 L/min/m2). Pulmonary arterial oxygen saturation declined significantly during exercise (rest 68%; exercise 31%) and during atrial pacing (control 72%; pacing 64%). The mean increment in pulmonary arterial pressure was 1.3 times greater with exercise (rest 14 mm Hg; exercise 20 mm Hg) than with pacing (control 12 mm Hg; pacing 16 mm Hg). Peak systemic arterial systolic pressure increased 14% with exercise but was unchanged by pacing. Systemic and pulmonary vascular resistances fell with exercise but changed insignificantly during atrial pacing. Stroke volume rose slightly with exercise but fell significantly with pacing.(ABSTRACT TRUNCATED AT 250 WORDS)