Thirty (10.8%) of 279 patients undergoing correction of a ventricular septal defect (VSD) from January, 1972, to September, 1986, also had a double-chambered right ventricle (DCRV). Age at operation ranged from 1.3 to 18.8 years (mean, 6.7 +/- 4.5 years [+/- standard deviation]). Seventeen patients were male, and 13 were female. Two-dimensional echocardiography was used after 1978 in the initial evaluation of 20 patients; however, the diagnosis of DCRV was made with the use of subcostal views only since 1984 in 4 of 5 patients. Surgical correction consisted of closure of the VSD and resection of anomalous muscle bundles through a right ventriculotomy (28 patients), and right atriotomy (2 patients). All patients survived and are asymptomatic 4.2 +/- 3.4 years following operation. Six patients have undergone catheterization postoperatively and 8 patients had intraoperative pressure recordings. The mean preoperative ratio of right ventricular to left ventricular pressures was 0.67 +/- 0.22 compared with 0.34 +/- 0.15 postoperatively (p less than .001). In 2 patients, DCRV was not recognized preoperatively or at VSD closure through a right atriotomy, and reoperation was necessary after DCRV was demonstrated at postoperative catheterization. DCRV may occur in approximately 10% of patients undergoing correction of VSD. Careful evaluation of echocardiographic and catheterization data preoperatively and careful evaluation of the anatomy intraoperatively are necessary so that DCRV not be overlooked, especially because most VSDs are now closed through the right atrium. Successful correction of VSD and DCRV is associated with excellent long-term results.