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 journalArticle

142 Citations (Scopus)

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

Fingerprint

Williams Syndrome
Sudden Death
Temperament
Coronary Stenosis
Physiologic Monitoring
Intellectual Disability
Cardiac Output
Myocardial Ischemia
Cardiac Arrhythmias
Autopsy
Cardiovascular Diseases
Growth

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., ... 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

Sudden death in Williams syndrome : Report of ten cases. / 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.

In: Journal of Pediatrics, Vol. 129, No. 6, 01.01.1996, p. 926-931.

Research output: Contribution to journalArticle

Bird, LM, Billman, GF, Lacro, RV, Spicer, RL, Jariwala, LK, Hoyme, HE, Zamora-Salinas, R, Morris, C, Viskochil, D, Frikke, MJ & Jones, MC 1996, 'Sudden death in Williams syndrome: Report of ten cases', Journal of Pediatrics, vol. 129, no. 6, pp. 926-931. https://doi.org/10.1016/S0022-3476(96)70042-2
Bird LM, Billman GF, Lacro RV, Spicer RL, Jariwala LK, Hoyme HE et al. Sudden death in Williams syndrome: Report of ten cases. Journal of Pediatrics. 1996 Jan 1;129(6):926-931. https://doi.org/10.1016/S0022-3476(96)70042-2
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. / Sudden death in Williams syndrome : Report of ten cases. In: Journal of Pediatrics. 1996 ; Vol. 129, No. 6. pp. 926-931.
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