open access

Vol 29, No 5 (2022)
Research Letter
Submitted: 2022-04-13
Accepted: 2022-05-04
Published online: 2022-07-08
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Heart valve disease in Hurler-Scheie syndrome

María del Carmen García del Rey12, Javier Castrodeza12, Ángel Pinto32, Maria Ángeles Espinosa Castro12, Cecilia Muñoz Delgado4, Francisco Fernández-Avilés12
·
Pubmed: 35818796
·
Cardiol J 2022;29(5):875-877.
Affiliations
  1. Cardiology Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain
  2. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain
  3. Cardiac Surgery Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain
  4. Internal Medicine Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain

open access

Vol 29, No 5 (2022)
Research letters — Clinical cardiology
Submitted: 2022-04-13
Accepted: 2022-05-04
Published online: 2022-07-08

Abstract

Not available

Abstract

Not available
Get Citation
Supp./Additional Files (3)
Supplemental Video 1. Four-chamber transoesophageal 0º view showing severe mitral regurgitation.
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96MB
Supplemental Video 2. Four-chamber MRI view showing a dilated left atrium, a normal sized and not hypertrophic left ventricle with preserved ejection fraction, normal right chambers and a lateral jet of severe mitral regurgitation.
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2MB
Supplemental Video 3. Surgical view of a myxomatous mitral valve in a patient with Hurler-Scheie syndrome with shortened and thickened chords accompanied by hypertrophic papillary muscles.
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83MB
About this article
Title

Heart valve disease in Hurler-Scheie syndrome

Journal

Cardiology Journal

Issue

Vol 29, No 5 (2022)

Article type

Research Letter

Pages

875-877

Published online

2022-07-08

Page views

3807

Article views/downloads

392

DOI

10.5603/CJ.a2022.0066

Pubmed

35818796

Bibliographic record

Cardiol J 2022;29(5):875-877.

Authors

María del Carmen García del Rey
Javier Castrodeza
Ángel Pinto
Maria Ángeles Espinosa Castro
Cecilia Muñoz Delgado
Francisco Fernández-Avilés

References (10)
  1. Brazier A, Hasan R, Jenkins P, et al. Urgent resection of a giant left atrial appendage aneurysm and mitral valve replacement in a complex case of Hurler-Scheie syndrome. BMJ Case Rep. 2015; 2015.
  2. Fischer TA, Lehr HA, Nixdorff U, et al. Combined aortic and mitral stenosis in mucopolysaccharidosis type I-S (Ullrich-Scheie syndrome). Heart. 1999; 81(1): 97–99.
  3. Pérez-López J, Morales-Conejo M, López-Rodríguez M, et al. Efficacy of laronidase therapy in patients with mucopolysaccharidosis type I who initiated enzyme replacement therapy in adult age. A systematic review and meta-analysis. Mol Genet Metab. 2017; 121(2): 138–149.
  4. Martins AM, Dualibi AP, Norato D, et al. Guidelines for the management of mucopolysaccharidosis type I. J Pediatr. 2009; 155(4 Suppl): S32–S46.
  5. Encarnacion CO, Hang D, Earing M, et al. Mucopolysaccharidoses causing valvular heart disease: report and review of surgical management. World J Pediatr Congenit Heart Surg. 2020; 11(4): NP22–NP24.
  6. Rocha RV, Alvarez RJ, Bermudez CA. Valve surgery in a mucopolysaccharidosis type I patient: early prosthetic valve endocarditis. Eur J Cardiothorac Surg. 2012; 41(2): 448–449.
  7. Robinson CR, Roberts WC. Outcome of combined mitral and aortic valve replacement in adults with mucopolysaccharidosis (the Hurler syndrome). Am J Cardiol. 2017; 120(11): 2113–2118.
  8. Rocha RV, Alvarez RJ, Bermudez CA. Valve surgery in a mucopolysaccharidosis type I patient: early prosthetic valve endocarditis. Eur J Cardiothorac Surg. 2012; 41(2): 448–449.
  9. Muenzer J, Wraith JE, Clarke LA. Mucopolysaccharidosis I: management and treatment guidelines. Pediatrics. 2009; 123(1): 19–29.
  10. Sherwood DJ, Adams MC, Mazzella AJ, et al. Mucopolysaccharidosis type i diagnosed by aortic and mitral valve replacement. JACC Case Rep. 2021; 3(18): 1891–1894.

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