The aim of this study was to evaluate an off-pump technique for total cavopulmonary connection. Between May 2000 and June 2004, 16 patients underwent total cavopulmonary connection without cardiopulmonary bypass. There were 8 males and 8 females, with a mean age of 9.8 +/- 6.2 years and a mean weight of 27.7 +/- 14.9 kg. Preoperative percutaneous oxygen saturation was 75% +/- 6%, and pulmonary arterial pressure was 12.6 +/- 2.7 mm Hg. Postoperative outcomes were retrospectively compared with a group of 17 patients who underwent total cavopulmonary connection with an extracardiac conduit under cardiopulmonary bypass. Two patients died early postoperatively. Chylothorax occurred in 2 patients and 3 underwent reoperation for fenestration between the extracardiac conduit and the right atrium in the early postoperative period. All survivors were acyanotic. On discharge from hospital, the percutaneous oxygen saturation breathing air was 95% +/- 3%. Patients in the off-pump group demonstrated significantly shorter extubation time and intensive care unit stay. This connection technique is easy to perform and helpful in the early management of patients with a functional univentricular heart. However, much remains to be learned about this unique physiologic system.