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The International Journal of Cardiovascular Imaging

Erschienen in:

17.10.2023 | Original Paper

Evaluation of cardiac findings in mucopolysaccharidosis

verfasst von: Bahar Dehghan, Noushin Rostampour, Mohsen Sedighi, Mohammad Hosseini Saryazdi, Mohammad Jokar Rizi, Neda Mostofizadeh, Mahin Hashemipour, Mehri Khoshhali

Erschienen in: The International Journal of Cardiovascular Imaging | Ausgabe 1/2024

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Abstract

Purpose

Mucopolysaccharidoses (MPS) are a group of rare genetic diseases and heart involvement is one of the important conflicts in most types, which may cause serious complications. We used M-Mode and two-dimensional speckle tracking echocardiography (2D-STE) to explore cardiovascular involvements in MPS patients.

Method

The present cross-sectional study investigated the frequency of cardiac involvements in MPS patients. Included participants were MPS types I, II, III, IV, and VI who underwent specialized echocardiography exams to assess valvular function, systolic and diastolic function, left ventricular ejection fraction (LVEF), and global longitudinal strain (GLS).

Results

35 patients were enrolled in this study. The total mean age of patients was 9.58 ± 5.11 years and 71.4% were male. Type IV (40%) and type III (31.4%) were the most frequent MPS. Although LVEF did not differ notably among MPS types, GLS was significantly different (p = 0.029). Mitral regurgitation was observed remarkably more in MPS type III (p = 0.001) while mitral stenosis was more common in type III (p = 0.007). There was a significant association between LVEF and GLS (β= -0.662; p = 0.025) and between LVEF and MPS type (β = 1.82; p = 0.025) when adjusted for GLS.

Conclusion

Cardiac complications are very common and are one of the most important causes of death in MPS patients. 2D-STE seems to be superior to M-Mode for detection of early and subclinical cardiac dysfunction in MPS patients.

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Titel: Evaluation of cardiac findings in mucopolysaccharidosis
verfasst von: Bahar Dehghan
Noushin Rostampour
Mohsen Sedighi
Mohammad Hosseini Saryazdi
Mohammad Jokar Rizi
Neda Mostofizadeh
Mahin Hashemipour
Mehri Khoshhali
Publikationsdatum: 17.10.2023
Verlag: Springer Netherlands
Erschienen in: The International Journal of Cardiovascular Imaging / Ausgabe 1/2024
Print ISSN: 1569-5794
Elektronische ISSN: 1875-8312
DOI: https://doi.org/10.1007/s10554-023-02983-y

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Evaluation of cardiac findings in mucopolysaccharidosis

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