Pulmonary Stenosis is a Predictor of Unfavorable Outcome After Surgery for Supravalvular Aortic Stenosis

Pediatric Cardiology, 04/10/2012

Pulmonary stenosis (PS) in patients with supravalvular aortic stenosis (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.


  • The authors identified all patients with SVAS from the 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 14.6years.
  • Overall mortality was 11.5 %.
  • One patient with preoperatively severely decreased LV–function died 27days postoperatively.
  • Two late deaths occurred 7 and 10years after the initial operation.
  • Reoperations were required in 4 patients (15 %), 4–19years 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).

Print Article Summary Cat 2 CME Report