Surgical management of valvular heart disease in mucopolysaccharidoses: a review of literature
File(s)
Published version
Author(s)
Rosser, Barbara
Chan, Calvin
Hoschtitzky, Andreas
Type
Journal Article
Abstract
Mucopolysaccharidoses are extremely rare diseases that are frequently presenting with structural heart problems of the aortic and mitral valve and myocardial dysfunction. In a substantial pro-portion this leads to heart failure and is a leading cause of death in these patients. As this gly-cosaminoglycan degradation defect is associated with other conditions strongly influencing the perioperative risk and choice of surgical technique, multidisciplinary planning is crucial to im-prove short- and long-term outcome. The extensive variance in clinical presentation between different impaired enzymes, and further within subgroups, calls for personalised treatment plans. Enzyme replacement therapies and bone marrow transplantation carry great potential as they may significantly abrogate the progress of the disease and as such reduce the clinical burden and im-prove life expectancy. Nevertheless, structural heart interventions may be required. We reviewed existing literature of the less than 50 published cases regarding surgical management, technique, and choice of prostheses. Although improvement in therapy has shown promising results in protecting valvar tissue when initiated in infancy, concerns regarding durability of this effect and durability of biological prostheses remain.
Date Issued
2022-02-04
Date Acceptance
2022-01-04
Citation
Biomedicines, 2022, 10 (2), pp.1-25
ISSN
2077-0383
Publisher
MDPI AG
Start Page
1
End Page
25
Journal / Book Title
Biomedicines
Volume
10
Issue
2
Copyright Statement
© 2022 by the authors.
Licensee MDPI, Basel, Switzerland.
This article is an open access article
distributed under the terms and
conditions of the Creative Commons
Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Licensee MDPI, Basel, Switzerland.
This article is an open access article
distributed under the terms and
conditions of the Creative Commons
Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
License URL
Identifier
https://www.mdpi.com/2227-9059/10/2/375
Subjects
1103 Clinical Sciences
Publication Status
Published
Date Publish Online
2022-02-04