Pediatric Cardiology

, Volume 14, Issue 2, pp 130–132 | Cite as

Severe mitral insufficiency in mucopolysaccharidosis type III-B (Sanfilippo syndrome)

  • Joseph Muenzer
  • Robert H. Beekman
  • Lisa M. Profera
  • Edward L. Bove
Case Reports


A 6-year-old girl with mucopolysaccharidosis (MPS) III-B (Sanfilippo syndrome) who developed severe mitral regurgitation and congestive heart failure requiring surgery (valvuloplasty) is reported. One year after surgery the patient remains well, with marked improvement in her physical activity, and without signs of heart failure. This is only the second report of severe mitral regurgitation in MPS III, and is the first report of a successful repair (valvuloplasty) of a dysplastic mitral valve in the MPS. Mitral valvuloplasty should be considered instead of valve replacement in any MPS patient with mitral valve regurgitation requiring surgery.

Key Words

Mucopolysaccharidoses Sanfilippo syndrome Mitral regurgitation Congestive heart failure Valvuloplasty 


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Copyright information

© Springer-Verlag New York Inc. 1993

Authors and Affiliations

  • Joseph Muenzer
    • 1
  • Robert H. Beekman
    • 2
  • Lisa M. Profera
    • 3
  • Edward L. Bove
    • 4
  1. 1.Division of Biochemical Genetics and MetabolismUniversity of Michigan Medical SchoolAnn ArborUSA
  2. 2.Division of CardiologyUniversity of Michigan Medical SchoolAnn ArborUSA
  3. 3.Department of Pediatrics, MSRB II 3570BUniversity of Michigan Medical CenterAnn ArborUSA
  4. 4.Section of Thoracic Surgery, Department of SurgeryUniversity of Michigan Medical SchoolAnn ArborUSA

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