Pulmonary stenosis is a predictor of unfavorable outcome after surgery for supravalvular aortic stenosis

Pediatr Cardiol. 2012 Oct;33(7):1131-7. doi: 10.1007/s00246-012-0267-y. Epub 2012 Mar 22.

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.

MeSH terms

  • Adolescent
  • Adult
  • Aortic Stenosis, Supravalvular / mortality
  • Aortic Stenosis, Supravalvular / surgery*
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Male
  • Postoperative Complications / diagnosis*
  • Postoperative Complications / mortality
  • Postoperative Complications / surgery
  • Pulmonary Valve Stenosis / diagnosis*
  • Pulmonary Valve Stenosis / mortality
  • Pulmonary Valve Stenosis / surgery
  • Reoperation
  • Risk Factors
  • Survival Rate
  • Treatment Outcome
  • Williams Syndrome / diagnosis
  • Williams Syndrome / mortality
  • Williams Syndrome / surgery