Clinical staging of Anderson-Fabry cardiomyopathy: An operative proposal.
Anderson-Fabry cardiomyopathy
Clinical management
Myocardial disease progression
Staging
Journal
Heart failure reviews
ISSN: 1573-7322
Titre abrégé: Heart Fail Rev
Pays: United States
ID NLM: 9612481
Informations de publication
Date de publication:
Mar 2024
Mar 2024
Historique:
accepted:
06
11
2023
medline:
18
3
2024
pubmed:
26
11
2023
entrez:
25
11
2023
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 38006470
doi: 10.1007/s10741-023-10370-x
pii: 10.1007/s10741-023-10370-x
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
431-444Commentaires et corrections
Type : ErratumIn
Informations de copyright
© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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