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

  • Meghan M. Chlebowski (a1) (a2), Bryce A. Heese (a2) (a3) and Lindsey E. Malloy-Walton (a1) (a2)

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.

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Corresponding author

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

References

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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: 11831197.
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: 170178.
3. Wraith, JE, Beck, M, Giugliani, R, Clarke, J, Martin, R, Muenzer, J. Initial report from the Hunter Outcome Survey. Genet Med 2008; 10: 508516.
4. Krovetz, J, Schiebler, G. Cardiovascular manifestations of genetic mucopolysaccharidoses. Birth Defects 1972; 8: 192196.
5. Okada, R, Rosenthal, IM, Scaravelli, G, Lev, M. A histopathologic study of the heart in gargoylism. Arch Pathol 1967; 84: 2030.

Keywords

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

  • Meghan M. Chlebowski (a1) (a2), Bryce A. Heese (a2) (a3) and Lindsey E. Malloy-Walton (a1) (a2)

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