Clinical staging of Anderson-Fabry cardiomyopathy : An operative proposal
© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature..
As a slowly progressive form of hypertrophic cardiomyopathy (HCM), Anderson-Fabry disease (FD) resembles the phenotype of the most common sarcomeric forms, although significant differences in presentation and long-term progression may help determine the correct diagnosis. A variety of electrocardiographic and imaging features of FD cardiomyopathy have been described at different times in the course of the disease, and considerable discrepancies remain regarding the assessment of disease severity by individual physicians. Therefore, we here propose a practical staging of FD cardiomyopathy, in hopes it may represent the standard for cardiac evaluation and facilitate communication between specialized FD centres and primary care physicians. We identified 4 main stages of FD cardiomyopathy of increasing severity, based on available evidence from clinical and imaging studies: non-hypertrophic, hypertrophic - pre-fibrotic, hypertrophic - fibrotic, and overt dysfunction. Each stage is described and discussed in detail, following the principle that speaking a common language is critical when managing such complex patients in a multi-disciplinary and sometimes multi-centre setting.
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:29 |
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| Enthalten in: |
Heart failure reviews - 29(2024), 2 vom: 15. März, Seite 431-444 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Del Franco, Annamaria [VerfasserIn] |
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| Links: |
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| Themen: |
Anderson-Fabry cardiomyopathy |
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| Anmerkungen: |
Date Completed 18.03.2024 Date Revised 18.03.2024 published: Print-Electronic ErratumIn: Heart Fail Rev. 2024 Jan 31;:. - PMID 38294670 Citation Status MEDLINE |
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| doi: |
10.1007/s10741-023-10370-x |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM364976950 |
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| 520 | |a © 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature. | ||
| 520 | |a As a slowly progressive form of hypertrophic cardiomyopathy (HCM), Anderson-Fabry disease (FD) resembles the phenotype of the most common sarcomeric forms, although significant differences in presentation and long-term progression may help determine the correct diagnosis. A variety of electrocardiographic and imaging features of FD cardiomyopathy have been described at different times in the course of the disease, and considerable discrepancies remain regarding the assessment of disease severity by individual physicians. Therefore, we here propose a practical staging of FD cardiomyopathy, in hopes it may represent the standard for cardiac evaluation and facilitate communication between specialized FD centres and primary care physicians. We identified 4 main stages of FD cardiomyopathy of increasing severity, based on available evidence from clinical and imaging studies: non-hypertrophic, hypertrophic - pre-fibrotic, hypertrophic - fibrotic, and overt dysfunction. Each stage is described and discussed in detail, following the principle that speaking a common language is critical when managing such complex patients in a multi-disciplinary and sometimes multi-centre setting | ||
| 650 | 4 | |a Journal Article | |
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| 650 | 4 | |a Anderson-Fabry cardiomyopathy | |
| 650 | 4 | |a Clinical management | |
| 650 | 4 | |a Myocardial disease progression | |
| 650 | 4 | |a Staging | |
| 700 | 1 | |a Iannaccone, Giulia |e verfasserin |4 aut | |
| 700 | 1 | |a Meucci, Maria Chiara |e verfasserin |4 aut | |
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| 700 | 1 | |a Olivotto, Iacopo |e verfasserin |4 aut | |
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