Aortic Dissection and Supravalvular Aortic Stenosis With Williams Syndrome Complicated by Infection

Williams syndrome (WS) is a contiguous gene syndrome involving the connective tissue, central nervous system, and cardiovascular disorders, such as supravalvular aortic stenosis (SVAS), pulmonary artery stenosis, and coronary artery anomaly, that carry a risk of sudden death. Although aortic dissect...

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Published inAnnals of thoracic surgery short reports Vol. 1; no. 4; pp. 674 - 678
Main Authors Matsunaga, Yoshikiyo, Suzuki, Hiroki, Shikata, Fumiaki, Honda, Takashi, Hirata, Yoichiro, Okamura, Toru, Ishikura, Kenji, Miyaji, Kagami
Format Journal Article
LanguageEnglish
Published Elsevier Inc 01.12.2023
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Summary:Williams syndrome (WS) is a contiguous gene syndrome involving the connective tissue, central nervous system, and cardiovascular disorders, such as supravalvular aortic stenosis (SVAS), pulmonary artery stenosis, and coronary artery anomaly, that carry a risk of sudden death. Although aortic dissection with SVAS in WS is extremely rare, it could be 1 of the triggers of sudden cardiac death. In this case, a patient with WS underwent ascending aorta replacement for aortic dissection that was potentially influenced by infective endocarditis and SVAS repaired by modified Brom aortoplasty.
ISSN:2772-9931
2772-9931
DOI:10.1016/j.atssr.2023.07.012