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Review
. 2022 Apr 4:9:851016.
doi: 10.3389/fcvm.2022.851016. eCollection 2022.

Pre-operative Considerations in Adult Mucopolysaccharidosis Patients Planned for Cardiac Intervention

Benjamin Cross  1 , Karolina M Stepien  2 , Chaitanya Gadepalli  3 , Ahmed Kharabish  4   5 , Peter Woolfson  6 , Govind Tol  7 , Petra Jenkins  1
Affiliations
Review

Pre-operative Considerations in Adult Mucopolysaccharidosis Patients Planned for Cardiac Intervention

Benjamin Cross et al. Front Cardiovasc Med. .

Abstract

Mucopolysaccharidoses (MPS) are rare lysosomal storage diseases characterized by multiorgan involvement and shortened longevity. Due to advances in therapies such as enzyme replacement therapy and haematopoietic stem cell therapy, life expectancy has increased posing newer challenges to patients and health professionals. One such challenge is cardiovascular manifestations of MPS, which can be life limiting and cause reduction in quality of life. Any cardiovascular intervention mandates comprehensive, multi-systemic work-up by specialist teams to optimize outcome. We highlight the importance of multidisciplinary evaluation of adult MPS patients requiring cardiovascular intervention. Clinical assessments and investigations are discussed, with a focus on the cardiac, anesthetic, airway, respiratory, radiological and psychosocial factors.

Keywords: adult MPS; cardiac surgery; glycosaminoglycans; heart disease; pre-operative assessment.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
(A–C) Magnetic resonance imaging, computed tomography, and coronary magnetic resonance imaging in MPS patients.
See this image and copyright information in PMC

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References

    1. Wraith JE. The mucopolysaccharidoses: a clinical review and guide to management. Arch Dis Child. (1995) 72:263-7. 10.1136/adc.72.3.263 - DOI - PMC - PubMed
    1. Braunlin EA, Harmatz PR, Scarpa M, Furlanetto B, Kampmann C, Loehr JP, et al. . Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management. J Inherit Metab Dis. (2011) 34:1183-97. 10.1007/s10545-011-9359-8 - DOI - PMC - PubMed
    1. Golda A, Jurecka A, Tylki-Szymanska A. Cardiovascular manifestations of mucopolysaccharidosis type VI (Maroteaux–Lamy syndrome). Int J Cardiol. (2012) 158:6-11. 10.1016/j.ijcard.2011.06.097 - DOI - PubMed
    1. Azevedo A, Schwartz IV, Kalakun L, Brustolin S, Burin MG, Beheregaray APC, et al. . Clinical and biochemical study of 28 patients with mucopolysaccharidosis type VI. Clin Genet. (2004) 66:208-13. 10.1111/j.1399-0004.2004.00277.x - DOI - PubMed
    1. Wraith JE, Beck M, Giugliani R, Clarke J, Martin R, Muenzer J, et al. . Initial report from the Hunter outcome survey. Genet Med. (2008) 10:508-16. 10.1097/GIM.0b013e31817701e6 - DOI - PubMed

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