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Pediatria pre prax 6/2022
Cardiovascular aspects of Williams-Beuren syndrome
Williams-Beuren syndrome is a rare, genetic, multisystemic disorder. The aim of this article is due to a case report to accentuate the potentially life-threatening clinical manifestations of this disease mainly in cardiovascular area. It also emphasizes the risk of cardiovascular collapse during interventions or investigations in general anaesthesia. The second aim of this article is to summarize general clinical signs of this disease, so that the patients with Williams-Beuren syndrome should be diagnosed as soon as possible and subsequently followed up and treated by a multidisciplinary medical team.
Keywords: Williams-Beuren syndrome, supravalvar aortic stenosis, supravalvar pulmonary stenosis, Coanda effect, cardiovascular collapse
