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European Journal of Pediatrics

, Volume 154, Issue 2, pp 98–101 | Cite as

Mitral and aortic regurgitation in 84 patients with mucopolysaccharidoses

  • C. -F. Wippermann
  • M. Beck
  • D. Schranz
  • R. Huth
  • I. Michel-Behnke
  • B. -K. Jüngst
Cardiology Original Paper

Abstract

In echocardiographic and necropsy studies nodular thickening of the mitral valve and, less frequently, of the aortic valve has been found in 60%–90% of patients with mucopolysaccharidoses (MPS). Little is known about the haemodynamic consequences of these morphological changes. In this study 84 unselected patients with different enzymatically proven MPS and 84 age and sex matched, healthy persons were studied prospectively by colour Doppler flow mapping. The patients' age ranged from 1 to 47 years (median 8.1 years). Mitral and aortic regurgitation were defined as a holosystolic or holodiastolic jet originating from the valve into the left atrium or the left ventricular outflow tract, respectively, with peak velocities exceeding 2.5 m/s. Of the 84 patients with satisfactory studies, mitral regurgitation was detected in 64.3% and aortic regurgitation in 40.5%, respectively. Regurgitation was severe in 4.8% of mitral valves and 8.3% of aortic valves. The frequency of aortic and/or mitral regurgitation was 75% in all patients, 89% in MPS I, 94% in MPS II, 66% in MPS III, 33% in MPS IV, and 100% in MPS VI. Combined mitral and aortic regurgitation was present in 29% of our patients. None of the control persons showed mitral or aortic regurgitation.

Key words

Mucopolysaccharidoses Mitral regurgitation Aortic regurgitation Doppler echocardiography 

Abbreviations

MPS IH

Hurler disease

MPS IS

Scheie disease

MPS II

Hunter disease

MPS III

Sanfilippo disease

MPS IV

Morquio disease

MPS VI

Lamy-Maroteaux disease

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Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • C. -F. Wippermann
    • 1
  • M. Beck
    • 1
  • D. Schranz
    • 1
  • R. Huth
    • 1
  • I. Michel-Behnke
    • 1
  • B. -K. Jüngst
    • 1
  1. 1.Children's HospitalJohannes Gutenberg UniversityMainzGermany

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