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a Department of Pediatric Cardiac Surgery, University Paris Descartes and Sick Children Hospital, Paris, France
b Department of Pediatric Cardiology, University Paris Descartes and Sick Children Hospital, Paris, France
Accepted for publication April 24, 2009.
* Address correspondence to Dr Vouhé, Chirurgie Cardiaque Pédiatrique, Hôpital Necker – Enfants Malades, 149 rue de Sèvres, Paris, 75015, France (Email: pascal.vouhe{at}nck.aphp.fr).
Background: Various surgical techniques have been proposed to repair supravalvular aortic stenosis. However, the optimal approach remains to be determined. The present study was undertaken to evaluate the results of surgery for supravalvular aortic stenosis and to compare the symmetric three-patch repair (Brom technique) with other surgical reconstructions.
Methods: Between 1995 and 2007, 34 patients with supravalvular aortic stenosis underwent surgery. Fourteen patients (41%) had Williams syndrome. Supravalvular stenosis was discrete in 24 patients (71%) and diffuse in 10 (29%). Eight patients (23%) underwent one-patch repair; 3 patients (9%) had inverted bifurcated patch enlargement (Doty repair) and 23 patients (68%) had symmetric repair (Brom three-patch technique). Follow-up was complete and ranged from 6 months to 12 years (mean, 5.8 years).
Results: There was one early and no late deaths. One patient (with one-patch repair) underwent reoperation for residual obstruction. At last follow-up, left ventricle to aorta peak gradient was 45 ± 28 mm Hg after one-patch repair, 30 ± 9 mm Hg after Doty operation, and 11 ± 18 mm Hg after symmetric Brom procedure. Brom repair was associated with a low incidence of residual obstruction (peak gradient
40 mm Hg) (2 of 22; 9.1%) and moderate aortic insufficiency (1 of 22; 4.5%).
Conclusions: Brom three-patch repair provides symmetric reconstruction of the aortic root in patients with supravalvular aortic stenosis. This may lead to improved midterm results in terms of relief of the obstruction and incidence of aortic insufficiency.
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