The clinical course was studied in 25 patients with ventricular septal defect (VSD) and aortic regurgitation (AR) who had undergone aortic valvuloplasty and VSD closure. Twelve patients had a doubly committed subarterial VSD and 13 had a perimembranous type of VSD. Preoperatively, progressive hemodynamic disturbance after the onset of AR occurred in 11 patients (44%). The follow-up period extended from 6 months to 23 years (mean 10 years). Four patients required prosthetic valve replacement after valvuloplasty. One of these had had initial improvement of the AR but required valve replacement 20 years later; the condition of the other 3 did not improve initially and their valves were replaced 1 month to 8.5 years later. Four other patients had no initial improvement as a result of valvular reconstructive surgery, but the AR did not progress and remained hemodynamically well tolerated. Hence, the overall success rate of the valvuloplasty, defined as improving or preventing progressive AR, was 21 of 25 (84%). Since initial and long-term improvement in aortic valve function can be expected in most patients after valvuloplasty and closure of the VSD, early surgical intervention is recommended in patients with a VSD and AR.