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Early childhood onset of high-grade atrioventricular block in Hunter syndrome

Part of: Electrophysiology

Published online by Cambridge University Press:  06 March 2018

Meghan M. Chlebowski Open the ORCID record for Meghan M. Chlebowski [Opens in a new window] ,
Bryce A. Heese  and
Lindsey E. Malloy-Walton
Meghan M. Chlebowski*
Affiliation:
Department of Pediatric Cardiology, Children’s Mercy, Kansas City, MO, USA Department of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA
Bryce A. Heese
Affiliation:
Department of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA Department of Pediatric Genetics, Children’s Mercy, Kansas City, MO, USA
Lindsey E. Malloy-Walton
Affiliation:
Department of Pediatric Cardiology, Children’s Mercy, Kansas City, MO, USA Department of Pediatrics, University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA
*
Author for correspondence: M. M. Chlebowski, MD, Division of Cardiology, Children’s Mercy Hospital, 2401 Gillham Road, Kansas City, MO 64108, USA. Tel: 816 234 3255; E-mail: mchlebowski@cmh.edu
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Abstract

Cardiac involvement has been reported in various mucopolysaccharidoses syndromes. Cardiac valve pathology is the most prominent cardiac manifestation of patients with these syndromes. To date, there have been no reports of early childhood onset of high-grade atrioventricular block in patients with Hunter syndrome. We present a case of a 3-year-old boy with Hunter syndrome who was found to have various degrees of atrioventricular block. This case highlights the importance of early routine cardiac screening for conduction abnormalities and close follow-up in patients with mucopolysaccharidoses syndromes.

Keywords

ChildrenHunter syndromeatrioventricular blockelectrophysiology (EP)

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 5 , May 2018 , pp. 786 - 787
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Copyright
© Cambridge University Press 2018 

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References

1. Braunlin, EA, Harmatz, PR, Scarpa, M, et al. Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management. J Inherit Metab Dis 2011; 34: 1183–1197.Google Scholar
2. Fesslova, V, Corti, P, Sersale, G, et al. The natural course and the impact of therapies of cardiac involvement in the mucopolysaccharidoses. Cardiol Young 2009; 19: 170–178.Google Scholar
3. Wraith, JE, Beck, M, Giugliani, R, Clarke, J, Martin, R, Muenzer, J. Initial report from the Hunter Outcome Survey. Genet Med 2008; 10: 508–516.Google Scholar
4. Krovetz, J, Schiebler, G. Cardiovascular manifestations of genetic mucopolysaccharidoses. Birth Defects 1972; 8: 192–196.Google Scholar
5. Okada, R, Rosenthal, IM, Scaravelli, G, Lev, M. A histopathologic study of the heart in gargoylism. Arch Pathol 1967; 84: 20–30.Google ScholarPubMed