Clinical course and outcomes of supravalvular aortic stenosis in adults

In adulthood, SVAS patients showed a stable clinical course without rapid progression. While cardiovascular events occurred in this young cohort, they were mostly obsereved in those with additional congenital heart defects, suggesting a more optimistic view for SVAS itself. No significant differences in outcomes were observed in patients with/without Williams-Beuren syndrome. Overall, SVAS tends to follow a more benign course in adulthood compared with childhood, potentially allowing for less intensive follow-up- though follow-up should still be individualised based on associated congenital heart defects and cardiovascular risks.

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Publication Details

Journal
Open Heart
Published
2025-01-01
DOI
https://doi.org/10.1136/openhrt-2025-003355
Primary Topic
Williams Syndrome Research
Type
article
Field-Weighted Citation Impact
0.00

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article

Clinical course and outcomes of supravalvular aortic stenosis in adults

Jolien W. Roos‐Hesselink, Monique R.M. Jongbloed, Roland R L van Kimmenade, Zoë A Keuning et al.
Open Heart
Williams Syndrome Research
article

Clinical course and outcomes of supravalvular aortic stenosis in adults

Jolien W. Roos‐Hesselink, Monique R.M. Jongbloed, Roland R L van Kimmenade, Zoë A Keuning, Berto J. Bouma, Adine R de Keijzer, Jolanda Kluin, Frederike Meccanici, Annemien E. van den Bosch, Joost P van Melle, Michiel Voskuil
article en

Abstract

In adulthood, SVAS patients showed a stable clinical course without rapid progression. While cardiovascular events occurred in this young cohort, they were mostly obsereved in those with additional congenital heart defects, suggesting a more optimistic view for SVAS itself. No significant differences in outcomes were observed in patients with/without Williams-Beuren syndrome. Overall, SVAS tends to follow a more benign course in adulthood compared with childhood, potentially allowing for less intensive follow-up- though follow-up should still be individualised based on associated congenital heart defects and cardiovascular risks.

Open HeartVol. 12(1)
Leiden University (NL), University Medical Center Groningen (NL), Radboud University Nijmegen (NL), Erasmus MC (NL), Radboud University Medical Center (NL), University Medical Center Utrecht (NL), ERN GUARD-Heart (NL), Erasmus University Rotterdam (NL)
Heart Foundation
Good health and well-being
Openalex Percentile: Top 87%
Williams Syndrome Research
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Clinical course and outcomes of supravalvular aortic stenosis in adults — Jolien W. Roos‐Hesselink, Monique R.M. Jongbloed, et al. · Open Heart (2025) | TGRS Research Map | TGRS