Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future.

Anderson-Fabry disease cardiac imaging cardiac involvement echocardiography multimodality imaging

Journal

Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588

Informations de publication

Date de publication:
06 May 2021
Historique:
received: 16 03 2021
revised: 21 04 2021
accepted: 26 04 2021
entrez: 2 6 2021
pubmed: 3 6 2021
medline: 3 6 2021
Statut: epublish

Résumé

Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Cardiac imaging plays a key role in the evaluation and management of Anderson-Fabry disease patients. The present review highlights the value and perspectives of standard and advanced cardiovascular imaging in Anderson-Fabry disease.

Identifiants

pubmed: 34066467
pii: jcm10091994
doi: 10.3390/jcm10091994
pmc: PMC8124634
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

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Auteurs

Roberta Esposito (R)

Department of Clinical Medicine and Surgery, Federico II University Hospital, 80122 Naples, Italy.
Mediterranea Cardiocentro, 80122 Naples, Italy.

Ciro Santoro (C)

Department of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, Italy.

Giulia Elena Mandoli (GE)

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, 53100 Siena, Italy.

Vittoria Cuomo (V)

Department of Clinical Medicine and Surgery, Federico II University Hospital, 80122 Naples, Italy.

Regina Sorrentino (R)

Department of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, Italy.

Lucia La Mura (L)

Department of Advanced Biomedical Sciences, Federico II University Hospital, 80131 Naples, Italy.

Maria Concetta Pastore (MC)

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, 53100 Siena, Italy.

Francesco Bandera (F)

Heart Failure Unit, Department of Cardiology, IRCCS Policlinico San Donato Milanese, University of Milan, 20122 Milan, Italy.

Flavio D'Ascenzi (F)

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, 53100 Siena, Italy.

Alessandro Malagoli (A)

Nephro-Cardiovascular Department Division of Cardiology, Baggiovara Hospital, University of Modena and Reggio Emilia, 41121 Modena, Italy.

Giovanni Benfari (G)

Division of Cardiology, Department of Medicine, University of Verona, 37132 Verona, Italy.

Antonello D'Andrea (A)

Division of Cardiology, Umberto I Hospital Nocera Inferiore (Salerno), Luigi Vanvitelli University, 81100 Caserta, Italy.

Matteo Cameli (M)

Division of Cardiology, Department of Medical Biotechnologies, University of Siena, 53100 Siena, Italy.

Classifications MeSH