Sudden death in Williams syndrome: Report of ten cases

L. M. Bird, G. F. Billman, R. V. Lacro, R. L. Spicer, L. K. Jariwala, H. E. Hoyme, R. Zamora-Salinas, C. Morris, D. Viskochil, M. J. Frikke, M. C. Jones

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Abstract

Williams syndrome (WS) is a recognizable pattern of malformation with mental retardation, mild growth deficiency, characteristic facies and temperament, and cardiovascular disease. Sudden death is a recognized complication of WS; however, it is thought to be rare. The clinical features of 10 children with WS who died suddenly are reported here, doubling the number of unexpected deaths reported in the literature. We suggest that sudden death is a more common complication than has been assumed previously. Pathologic findings on the seven autopsy cases implicate two anatomic abnormalities that predispose individuals with WS to sudden death: coronary artery stenosis and severe biventricular outflow tract obstruction. The mechanisms for sudden death for both anatomic subgroups include myocardial ischemia, decreased cardiac output, and arrhythmia. We believe these observations warrant the development of strategies for monitoring patients with WS in an attempt to identify those at increased risk of sudden death.

Original languageEnglish (US)
Pages (from-to)926-931
Number of pages6
JournalJournal of Pediatrics
Volume129
Issue number6
DOIs
StatePublished - Jan 1 1996

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ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Bird, L. M., Billman, G. F., Lacro, R. V., Spicer, R. L., Jariwala, L. K., Hoyme, H. E., Zamora-Salinas, R., Morris, C., Viskochil, D., Frikke, M. J., & Jones, M. C. (1996). Sudden death in Williams syndrome: Report of ten cases. Journal of Pediatrics, 129(6), 926-931. https://doi.org/10.1016/S0022-3476(96)70042-2