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

Research output: Contribution to journalArticlepeer-review

168 Scopus citations


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
Issue number6
StatePublished - 1996
Externally publishedYes

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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