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Shiohama T, Fujii K, Ebata R, et al. Coronary ostium occlusion by coronary cusp displacement in Williams syndrome. Pediatr Int. 2015;58(6):487-490doi: 10.1111/ped.12828.
Shiohama, T., Fujii, K., Ebata, R., Funabashi, N., Matsumiya, G., Saito, Y. K., Takechi, F., Yonemori, Y., Nakatani, Y., & Shimojo, N. (2016). Coronary ostium occlusion by coronary cusp displacement in Williams syndrome. Pediatrics international : official journal of the Japan Pediatric Society, 58(6), 487-490. https://doi.org/10.1111/ped.12828
Shiohama, Tadashi, et al. "Coronary ostium occlusion by coronary cusp displacement in Williams syndrome." Pediatrics international : official journal of the Japan Pediatric Society vol. 58,6 (2016): 487-490. doi: https://doi.org/10.1111/ped.12828
Shiohama T, Fujii K, Ebata R, Funabashi N, Matsumiya G, Saito YK, Takechi F, Yonemori Y, Nakatani Y, Shimojo N. Coronary ostium occlusion by coronary cusp displacement in Williams syndrome. Pediatr Int. 2016 Jun;58(6):487-490. doi: 10.1111/ped.12828. Epub 2015 Dec 29. PMID: 26711184.
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Pediatr Int. 2016 Jun;58(6):487-490. doi: 10.1111/ped.12828. Epub 2015 Dec 29.

Coronary ostium occlusion by coronary cusp displacement in Williams syndrome.

Pediatrics international : official journal of the Japan Pediatric Society

Tadashi Shiohama, Katsunori Fujii, Ryota Ebata, Nobusada Funabashi, Goro Matsumiya, Yuko Kazato Saito, Fumie Takechi, Yoko Yonemori, Yukio Nakatani, Naoki Shimojo

Affiliations

  1. Departments of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan.
  2. Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine, Chiba, Japan.
  3. Department of Cardiovascular Surgery, Chiba University Graduate School of Medicine, Chiba, Japan.
  4. Department of Diagnostic Pathology, Chiba University Graduate School of Medicine, Chiba, Japan.

PMID: 26711184 DOI: 10.1111/ped.12828

Abstract

Williams syndrome is a contiguous gene deletion syndrome resulting from a heterozygous deletion on chromosome 7q11.23, and is characterized by distinctive facial features and supravalvular aortic stenosis (SVAS). This syndrome rarely presents unpredictable cardiac death, and yet, as illustrated in the present case, it is still not possible to predict it, even on close monitoring. We herein describe the case of a 6-year-old Japanese girl with Williams syndrome, who had sudden cardiac collapse due to cardiac infarction after pharyngitis. Cardiac failure followed a critical course that did not respond to catecholamine support or heart rest with extracardiac mechanical support. Although marked coronary stenosis was not present, the left coronary cusp abnormally adhered to the aortic wall, which may synergistically cause coronary ostium occlusion with SVAS. Altered hemodynamic state, even that caused by the common cold, may lead to critical myocardial events in Williams syndrome with SVAS.

© 2015 Japan Pediatric Society.

Keywords: Williams syndrome; extracorporeal membrane oxygenation; sudden cardiac collapse; supravalvular aortic stenosis

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