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The cardiac conduction system in Hurler syndrome: pathological features and clinical implications

Published online by Cambridge University Press:  19 August 2008

Louis Tsun-cheung Chow  and
Wing-Hing Chow
Louis Tsun-cheung Chow*
Affiliation:
From the Institute of Pathology, Queen Elizabeth Hospital, Hong Kong
Wing-Hing Chow
Affiliation:
Department of Medicine, Grantham Hospital, Hong Kong
*
1Dr.Louis T.C.Chow, Institute of Pathology, Queen Elizabeth Hospital, Wylie Road, Kowloon, Hong Kong. Tel. 852-710-2816; Fax. 852-385-2455.
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Summary

We studied the cardiac conduction system in a case of Hurler syndrome. There was dense fibrosis of the supporting matrix of the sinus node and accumulation of mucopolysaccharide in the nodal cells. The bundle branches showed prominent hydropic degeneration, being encased and punctuated by dense fibrous tissue. These changes in the conduction system may predispose to the development of arrhythmias, accounting for the sudden deaths in Hurler syndrome.

Keywords

MucopolysaccharidosisHurler syndrome heartcardiac conduction system
Type
Brief Reports
Information
Cardiology in the Young , Volume 2 , Issue 2 , April 1992 , pp. 196 - 199
Copyright
Copyright © Cambridge University Press 1992

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