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The Ross procedure is the procedure of choice for congenital aortic valve disease.

OBJECTIVES: The Ross procedure has emerged as an attractive option for aortic valve replacement in children and young adults. Our objective was to review our experience with the Ross procedure in young patients with congenital aortic valve disease. We also sought for evidence of growth in the autograft.

METHODS: From January 1990 to July 2000, 260 patients underwent the Ross procedure for various aortic valve diseases. There were 136 patients less than 18 years of age. Fifty-three (38%) of these patients had congenital aortic valve disease. Ages ranged from 3 months to 18 years (mean, 8 plus minus 5 years; median, 9 years). Ten patients were less than 2 years of age. Pure aortic stenosis was present in 18 patients, mixed stenosis and regurgitation in 32, and pure aortic regurgitation in 3. The aortic valve was bicuspid in 29 patients. Twenty-nine patients had previous procedures, mostly balloon dilation of the aortic valve (n = 8) or surgical aortic valvotomy (n = 12).

RESULTS: In all patients immediate results demonstrated a normally functioning neoaortic valve with not more than trivial aortic valve regurgitation. In the patients with stenosis, all levels of obstruction were relieved, and the gradient across the left ventricular outflow tract was completely abolished. Hospital mortality was 3 (5.6%) of 53 (overall Ross mortality was 34 of 260 [1.5%]). The patients were followed up for a mean of 4 years and up to 10 years. One patient died late of a noncardiac cause. Actuarial survival at 10 years was 94% plus minus 2%, and freedom from all events was 93% plus minus 5%. Only 1 patient needed autograft replacement for endocarditis. Intervention related to right ventricle-pulmonary artery conduit was required in 3 patients: balloon dilatation in 2, and reoperation in 1. At last follow-up, all patients but one were classified as being in New York Heart Association functional class I or II with normal or near-normal autograft valve function. Serial measurement of the left ventricular outflow tract and aortic root showed that as patients grew, the size of the outflow tract increased. When indexed to body surface area, this increase correlated with the patients' expected somatic growth.

CONCLUSIONS: The Ross procedure for congenital aortic valve disease in children and young adults offers excellent hemodynamics, with the added advantage of real potential for growth. It should be considered the treatment of choice in this age group.

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