Long follow-up (to 43 years) of ventricular septal defect with audible aortic regurgitation

From 1946 to March 1989, 92 patients (33 women and 59 men) were seen with ventricular septal defect (VSD) and audible aortic regurgitation (AR). The VSD was subcristal in 62 patients, subpulmonary in 21 and unknown in the remaining 9. The median age of onset of AR was 5.3 years. The risk of developing AR was 2.5 times greater in those with a subpulmonary VSD. The aortic valve was tricuspid in 90% and bicuspid in 10%. Prolapse was seen in 90% of those with subcristal VSD and in all with subpulmonary VSD. Pulmonary stenosis was seen in 46% of the patients with gradients ranging from 10 to 55 mm Hg. The incidence of infective endocarditis was 15 episodes/1,000 patient years. Among 20 patients followed medically, for 297 patient years, 1 died (1959) and most have been stable, including 2 followed for greater than 30 years. In the 72 patients operated on, there were 15 perioperative and 5 late deaths. Operations consisted of VSD closure alone in 7, VSD closure and valvuloplasty in 50 and VSD closure and aortic valve replacement in the other 15. Valvuloplasty was more effective in those operated on under age 10 compared to those older than 15 years (46 vs 14%). The durability of the valvuloplasty was 76% at 12 years and 51% at 18 years.