Congenital aortic valve disease. Improved survival and quality of life.

OBJECTIVE: The purpose of the study was to assess the effect of recent trends in surgical management, including use of the Ross Operation, on improved survival and quality of life in patients treated surgically for aortic valve (AV) disease at Oklahoma Children's Hospital.
BACKGROUND: Surgical treatment of congenital AV disease has proved to be palliative, but newer procedures may be improving outcomes.
METHODS: A retrospective review of 301 patients, age 1 day to 26 years (median, 5 years), having a surgical AV procedure or aortic balloon valvuloplasty at Children's Hospital of Oklahoma between 1960 and February 1996, was conducted. Information was collected on all prior and subsequent operations, and follow-up within 1 year was 96% complete.
RESULTS: Survival for all patients was 90% +/- 2% at age 10 years and 73% +/- 8% at age 25. By age 5, 52% +/- 4% had required an AV procedure, 89% +/- 3% by age 15. Patient survival was affected adversely by the diagnosis of valvar aortic stenosis, 79% +/- 6% at age 25 compared to 95% +/- 4% for subvalvar aortic stenosis or aortic insufficiency (p = 0.01). The AV morphology did not affect survival, but patients with a bicuspid or unicuspid valve required operative intervention at an earlier age. Survival after autograft replacement of the AV (Ross Operation) was significantly better than for other types of valve replacement (p = 0.0043). Quality of life as assessed by need for reoperation favors the use of the Ross Operation, with freedom from reoperation at 9 years of 87% +/- 7% compared to 55% +/- 5% in all patients after first AV surgery (p = 0.003).
CONCLUSIONS: The Ross Operation appears to have a significant advantage in survival and quality of life in children requiring a valve replacement as a first operation or after a prior AV procedure.