title: Pulmonary stenosis is a predictor of unfavorable outcome after surgery for supravalvular aortic stenosis.
abstract: We 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.
authors.affiliation: Department of Cardiovascular Surgery, Deutsches Herzzentrum München an der Technischen Universität München, 80636, Munich, Germany. samprec@dhm.mhn.de
authors.forename: Jelena
authors.initials: J
authors.lastname: Kasnar-Samprec
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hörer
authors.forename: Hanna
authors.initials: H
authors.lastname: Bierwirth
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Prodan
authors.forename: Julie
authors.initials: J
authors.lastname: Cleuziou
authors.forename: Andreas
authors.initials: A
authors.lastname: Eicken
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Lange
authors.forename: Christian
authors.initials: C
authors.lastname: Schreiber
mesh_terms: D000293:Adolescent; D000328:Adult; D021921:Aortic Stenosis, Supravalvular / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D012086:Reoperation; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome; D018980:Williams Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery