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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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References

1.Neufeld, EF, Nuenzer, J. The mucopolysaccharidoses. In: Scriver, CR, Beaudet, AL, Sly, WS, Valle, D (eds). The metabolic basis of inherited disease, McGraw-Hill, New York, 1989, pp 1565–1587.Google Scholar
2.Buist, NRM, Kennaway, NG, Kelly, TE, Lloyd, J, Fernandes, J, Hume, R, Forfar, JO. Metabolic disorders. In: Forfar, JO, Arneil, GC (eds). Textbook of Paediatrics. Churchill Livingstone, Edinburgh, 1984, pp 1259–1266.Google Scholar
3.Lindsay, S. The cardiovascular system in gargoylism. Br Heart J 1950; 12: 17–32.CrossRefGoogle ScholarPubMed
4.Krovetz, LJ, Lorincz, AE, Schiebler, GL. Cardiovascular mani festations of the Hurler syndrome. Hemodynamic and angiocardiographic observations in 15 patients. Circulation 1965; 31: 132–141.CrossRefGoogle Scholar
5.Nelson, J, Shields, MD, Mulholland, HC. Cardiovascular studies in the mucopolysaccharidoses. J Med Genet 1990; 27: 94–100.CrossRefGoogle ScholarPubMed
6.Ferrans, VJ, Boyce, SW. Metabolic and familial diseases. In Silver, ND (ed). Cardiovascular Pathology. Churchill Living-stone, New York, 1983, pp 948–954.Google Scholar
7.Lagunoff, Gritzka, TL. The site of mucopolysaccharide accumulation in Hurler's syndrome. An electron microscopic and histochemical study. Lab Invest 1966; 15: 1578–1588.Google Scholar
8.Okada, R, Rosenthal, IM, Scaravelli, G, Lev, N. A histopathologic study of the heart in gargoylism. Arch Pathol 1967; 84: 20–30.Google ScholarPubMed
9.Renteria, VG, Ferrans, VJ, Roberts, WC. The heart in the Hurler Syndrome. Gross, histologic and ultrastructural obser vation in five necropsy cases. Am J Cardiol 1976; 38: 487–501.Google Scholar
10.Davies, MJ, Anderson, RH, Becker, AE. The conduction system of the heart. Butterworths, London, 1983.Google Scholar