From May 1981 to September 1995, 38 patients received a superior vena cava-pulmonary artery anastomosis in association with biventricular repair. Patients were divided into four groups on the basis of indication for operation. Group A (19 patients) had a small physiologic right ventricle defined by tricuspid anulus z values or predicted right ventricular volume. Group B (11 patients) had a functionally compromised right ventricle. Group C (four patients) consisted of those receiving a superior vena cava-pulmonary artery anastomosis as a facilitation to biventricular repair. Group D (four patients) was defined by acute postoperative right ventricular dysfunction. Age ranged from 5 months to 51 years (median 3.5 years). There were 14 different underlying primary diagnoses in this cohort and multiple associated anomalies. Operative mortality was as follows: group A, two of 19 (10.5%); group B, two of 11 (18%); group C, none of four (0%); and group D, three of four (75%). Follow-up is complete in 37 of 38 patients (97%), ranging from 1 to 174 months (mean 46.3 +/- 36.9). Twenty-two patients are in New York Heart Association functional class I and eight patients are in class II. No clinical evidence of cyanosis or protein-losing enteropathy has been detected. With the use of this adjunctive approach, acceptable intermediate-term outcomes were obtained in patients having an anatomically or functionally compromised pulmonary ventricle. The anastomosis safely facilitates repair in a subset of patients. Results for this procedure when used as a salvage operation for right ventricular dysfunction have not been satisfactory.