The application of all-autologous three-sinus repair for supravalvular pulmonary stenosis

Abstract

Various surgical techniques have been proposed for the repair of supravalvular pulmonary stenosis (SVPS) in pediatric populations. Whereas growth potential should be promised, excessive expansion under the presence of undiminished high pulmonary arterial pressure should be avoided. The authors applied all-autologous three-sinus repair, the so-called modified Brom or Myers technique, to SVPS and examined the midterm outcomes. Between March 2010 and March 2012, 15 patients (8 boys) with a median age of 12 months who had SVPS underwent all-autologous three-sinus repair. Of the 15 patients, 13 (87 %) had previously undergone pulmonary artery (PA) banding for treatment of high pulmonary vascular resistance. Two patients (13 %) had Noonan syndrome. A follow-up evaluation was completed for all the patients, and the median follow-up period was 13.5 months (range, 1 month to 2.4 years). No mortalities occurred. The diameter of the stenotic part at the main PA increased from 47.0 ± 14.1 % (range 29.1-70.0 %) of the normal PA diameter at the preoperative evaluation to 108.9 ± 25.7 % (range 58.9-148.1 %) at 6 months, and then to 104.7 ± 11.4 % (range 87.7-134.1 %) 1 year after the operation. The estimated mean pressure gradient across the main PA decreased from 76.2 ± 12.2 mmHg (range 57.8-108.2 mmHg) at the preoperative evaluation to 11.3 ± 12.0 mmHg (range 1.4-49.0 mmHg) at 6 months, and then to 6.4 ± 5.5 mmHg (range 2.0-19.4 mmHg) 1 year after the operation. No patients showed moderate or greater pulmonary insufficiency. The midterm outcomes after supravalvular pulmonary stenosis by all-autologous three-sinus repair were acceptable. Although a long-term follow-up evaluation is mandatory, application of this technique may provide a just enough growth of the reconstructed main pulmonary artery with symmetric pulmonary valve geometry.