Abstract
The congenitally bicuspid aortic valve is the most common etiologic factor associated with clinically significant aortic stenosis and/or regurgitation in pediatric patients. Beyond infancy, surgical intervention typically involves valve repair with cusp thinning and commissurotomy or valve replacement, primarily with pulmonary autograft in the current era. An aortic valve repair technique using tricuspidization with cusp extension was introduced in 1999. This study compares the midterm clinical outcome in patients undergoing valve repair by tricuspidization with cusp extension with those receiving a pulmonary autograft (Ross). A retrospective study was performed on all consecutive patients with symptomatic bicuspid aortic valve disease who underwent tricuspidization with cusp extension or a Ross procedure between 1999 and 2005. In both groups, all patients were at least 1 year of age at time of the operation. During this period, 21 children (median age 12.6 years, range 2.6-18 years) underwent tricuspidization with cusp extension (TCE group) and 25 children (median age 10.2 years, range 11.5 months-20.1 years) underwent the Ross procedure. Prior balloon valvuloplasty was performed in 5 (24%) of the children in the TCE group and 16 (64%) of the children in the Ross group. Prior surgical commissurotomy was performed in 4 (19%) TCE patients and in 9 (36%) Ross patients. During a median follow-up period of 36.4 months (range 2.5 months-7.4 years), 2 (10%) patients in the TCE group required valve-preserving early revision of the repair, 2 (10%) TCE patients required subsequent aortic valve replacement at 16 and 33 months, 1 (4%) Ross patient required subsequent valve repair at 5 years, and 1 (4%) Ross patient underwent cardiac transplantation at 46 months. At 36 months, the actuarial freedom from reintervention on the aortic valve or autograft was 90% in the TCE group, with 11 patients at risk, and 100% in Ross patients, with 13 patients at risk (P = .39); the freedom from moderate valve dysfunction or reintervention was 66% for TCE patients and 95% for Ross patients (P = .07). There were no deaths, and all but 1 Ross patient remain in New York Heart Association class I. Reintervention rates in patients undergoing tricuspidization with cusp extension or a primary Ross procedure are similar. Valve performance in the TCE group is satisfactory at midterm follow-up, but the Ross repair appears to provide greater stability of valve function. These results suggest that repair with valve tricuspidization and cusp extension provides reliable palliation of the symptomatic bicuspid aortic valve.
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