Eight anesthetized dogs underwent closure of the tricuspid valve and a Fontan procedure, and the right ventricular cavity was reduced in stepwise fashion. There was an increase in right atrial pressure from 9.3 +/- 2.2 to 14.1 +/- 2.4 mm Hg (p less than 0.001), a decrease in pulmonary artery pulse pressure from 10.8 +/- 2.2 to 6.8 +/- 2.2 mm Hg (p less than 0.01), and a decrease in cardiac index from 2.7 +/- 0.3 to 2.2 +/- 0.2 L/min/m2 (p less than 0.001) when the ventricular size was dropped from 50% to 25% of normal. The difference between mean pulmonary artery pressure and mean right atrial pressure, which reflects the positive stroke work index of the ventricle, disappeared once the right ventricular cavity was reduced to 25% of normal (15.0 +/- 6.1 versus 14.1 +/- 2.4 mm Hg; p = not significant). Experimental results were correlated with postoperative catheterization data from 19 patients with tricuspid atresia who had the Fontan operation. Mean right atrial pressure was 18 +/- 4.6 mm Hg and cardiac index was 2.35 +/- 0.65 L/min/m2 in patients with a direct atrium-pulmonary artery anastomosis or an atrioventricular anastomosis with a right ventricular cavity less than 30% of normal versus 13 +/- 3.2 mm Hg and 3.42 +/- 0.46 L/min/m2 for those with an atrioventricular connection and a right ventricular cavity greater than 30% of normal (p less than 0.05 and p less than 0.02, respectively). The right ventricle enlarged from 27% +/- 6% of normal preoperatively to 35% +/- 10% of normal on follow-up (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)