Short and Midterm Results of Aortic Valve Cusp Extension in the Treatment of Children With Congenital Aortic Valve Disease

Background We evaluated our experience with aortic valve cusp extension techniques to identify predictors of successful intraoperative repair and subsequent durability. Methods Twenty-two children (ages 5–18 years) underwent aortic cusp extension with autologous pericardium between 1999 and 2005. Sixteen children had previous surgical or percutaneous intervention. Ten children had bicuspid aortic valves. Cusp extensions were performed on 1 cusp in 3 patients, 2 cusps in 3, and 3 cusps in 16. Serial echocardiographic measures (n = 81) were obtained during a 5-year period and underwent blinded review. Longitudinal trajectories of ventricular and aortic valve function were modeled using mixed linear regression analysis. Results There was no hospital or late mortality. Five-year freedom from valve replacement was 75%. Comparison of preoperative and post-repair echocardiograms demonstrated reductions in aortic insufficiency (decreased in jet-width/aortic valve diameter ratio from 0.39 ± 0.12 to 0.22 ± 0.11; p < 0.0001), aortic stenosis (decreased in peak aortic valve gradient from 41 ± 25 mm Hg to 29 ± 15 mm Hg; p = 0.04), and left ventricular end-diastolic dimensions Z-score (decreased from 1.39 ± 0.38 to 1.16 ± 0.34; p < 0.001). During the follow-up period, post-repair jet-width and aortic valve diameter increased nonlinearly (p < 0.001). Patients with postoperative peak aortic gradients greater than 30 mm Hg had progression of aortic stenosis, whereas those with lesser postoperative peak gradients tended to regress during follow-up (p < 0.001). The decrement in Z-score of the left ventricular end-diastolic dimensions remained stable during the follow-up period. Conclusions Aortic valve cusp extension can result in acceptable hemodynamic results with stabilization of left ventricular geometry. However, residual lesions are common and progression and regression of these lesions can be predicted based on echocardiographic data.