Cardiac involvement in mucopolysaccharidoses

Kadir Babaoǧlu, Gamze Özsoy, Köksal Binnetoǧlu

Research output: Contribution to journalArticlepeer-review

Abstract

Introduction: Mucopolysaccharidoses (MPS) are an important group of lysosamal storage diseases. Commonly reported cardiac involvements are mitral leaflet thickening and accompanying prolapsus, regurgitation and rarely stenosis. In this study, it was aimed to evaluate cardiac in involvements in patients with MPS. Materials and Method: We studied a total of 11 children with MPS who were admitted to our department between 2005 and 2009. Cardiac status was evaluated retrospectively with telecardiography, electrocardiography and echocardiography. Results: The ages of the patients (6 girls and 5 boys) ranged between 1.5-14 years and the mean age was 7.44±5.35 (median 7) years. Each of Type II and IV were identified in three patients; type I in two and type VI in one. In two patients, MPS type has not been identified yet. Thickening of the mitral valve with or without regurgitaion and prolapsus was the most common lesion seen in all patients. Additional involvement of the aortic valve was detected in 4 (36.3%) and additional involvement of tricuspid valve in 2 patients. Septal hypertrophy was found in one patient and pulmonary hypertension in another. No arrhythmia was found in any patient. Conclusion: Cardiac involvement is frequent in MPS. Mitral valve deformation is the most frequent finding. Echocardiographic examination should be performed periodically even if the patient has no clinical signs of cardiac disease.

Original languageEnglish (US)
Pages (from-to)48-51
Number of pages4
JournalGuncel Pediatri
Volume8
Issue number2
StatePublished - 2010
Externally publishedYes

Keywords

  • Cardiac diseases
  • Children
  • Echocardiography
  • Mucopolysaccharidoses

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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