Surgical Correction of Supravalvar Aortic Stenosis: 52 Years' Experience

Rosa Roemers, Jolanda Kluin, Frederiek de Heer, Sara Arrigoni, Regina Bokenkamp, Joost van Melle, Tjark Ebels, Mark Hazekamp*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Objectives: Supravalvar aortic stenosis (SVAS) is a rare congenital anomaly. The "single-patch technique," "'two sinus augmentation with an inverted Y-patch" (both nonsymmetrical corrections), "three-patch technique," and the "slide aortoplasty" (both symmetrical corrections) are the techniques implemented by the majority of surgeons for the correction of SVAS. In the few studies that compared these techniques, no technique was shown to be superior over another. The aim of the present study is to review the 52-year experience with the surgical correction of SVAS in two of four congenital cardiothoracic surgical centers in the Netherlands. Methods: We retrospectively reviewed all patient files of those who underwent an operation to correct their SVAS, between 1962 and 2014 in our centers. Patients were divided according to their operating technique. These groups were compared using the end points freedom from reoperation and mortality. Results: A total of 49 patients were included, 23 (46.9%) patients in the nonsymmetrical group and 26 (53.1%) patients in the symmetrical group. Survival after 20 years in the nonsymmetrical group was 80% (standard error [SE]: 0.091) and in the symmetrical group was 85% (SE: 0.085; P = .163). Freedom from reoperation after 20 years in the nonsymmetrical group was 88% (SE: 0.079) and in the symmetrical group was 71% (SE: 0.107; P = 0.313). Conclusion: In this patient group, there is no significant difference in survival and freedom from reoperation between the different surgical techniques for SVAS repair. Compared to the survival in the general population, the survival of SVAS patients is remarkably low. Apparently, SVAS is not a benign disease and probably patients should be followed more closely for the rest of their lives.

Original languageEnglish
Pages (from-to)131-138
Number of pages8
JournalWorld journal for pediatric and congenital heart surgery
Volume9
Issue number2
DOIs
Publication statusPublished - Mar-2018

Keywords

  • supravalvar aortic stenosis
  • supravalvular aortic stenosis
  • Williams syndrome
  • three-patch technique
  • 3-PATCH TECHNIQUE
  • ELASTIN GENE
  • REPAIR
  • OUTCOMES
  • CHILDREN
  • AORTOPLASTY

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