• Jelena Kasnar-Samprec, Jürgen Hörer, Hanna Bierwirth, Zsolt Prodan, Julie Cleuziou, Andreas Eicken, Rüdiger Lange, and Christian Schreiber.
• Department of Cardiovascular Surgery, Deutsches Herzzentrum München an der Technischen Universität München, 80636, Munich, Germany. samprec@dhm.mhn.de
• Pediatr Cardiol. 2012 Oct 1; 33 (7): 1131-7.

AbstractWe sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100 %) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65 %) were diagnosed with Williams-Beuren-Syndrome, six (17 %) had a diffuse form of SVAS and 10 (39 %) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p = 0.30) or diffuse form of SVAS (p = 0.13). Patients with PS were operated at younger age (p = 0.028). Median follow-up time was 14.6 years. Overall mortality was 11.5 %. One patient with preoperatively severely decreased LV-function died 27 days postoperatively. Two late deaths occurred 7 and 10 years after the initial operation. Reoperations were required in 4 patients (15 %), 4-19 years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p = 0.005) and for the combined reoperation/death end-point (p = 0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.

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