Between May 1974 and June 1990, 28 double chambered right ventricle underwent surgical repair. Twenty-one (75%) had high obstructive anomalous muscle bundles and in seven (25%) the anomalous muscle bundles were in low position. A ventricular septal defect (VSD) was present in all cases and a subaortic stenosis in six. Longitudinal right ventriculotomy was performed in 15 (53.57%) patients and a combined pulmonary arteriotomy and right atriotomy was used in 13 (46.43%). In the group treated by ventriculotomy 2 patients died shortly after operation (one as a consequence of disseminated infection and one after a lung lobectomy); another patient developed patch dehiscence and reoperation was performed via right atrium a year after. In other 3 patients the Doppler revealed a residual gradient of 20 to 30 mmHg. There were no complications in the group of patients that underwent right atriotomy. In addition to resection of the anomalous muscle bundles, the associated cardiac anomalies were corrected in all patients. Follow-up of 5.5 +/- 3.8 years after operation showed that 26 (92.86%) patients were alive and asymptomatic. We conclude that double chambered right ventricle can be early repaired with good results using a combined procedure with right atriotomy and pulmonary arteriotomy.