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 glycosphin-golipids 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.

Cardiac imaging in anderson-fabry disease: Past, present and future / R. Esposito, C. Santoro, G.E. Mandoli, V. Cuomo, R. Sorrentino, L. La Mura, M.C. Pastore, F. Bandera, F. D'Ascenzi, A. Malagoli, G. Benfari, A. D'Andrea, M. Cameli. - In: JOURNAL OF CLINICAL MEDICINE. - ISSN 2077-0383. - 10:9(2021), pp. 1994.1-1994.16. [10.3390/jcm10091994]

Cardiac imaging in anderson-fabry disease: Past, present and future

F. Bandera;
2021

Abstract

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 glycosphin-golipids 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.
No
English
Anderson-Fabry disease; Cardiac imaging; Cardiac involvement; Echocardiography; Multimodality imaging
Settore MED/11 - Malattie dell'Apparato Cardiovascolare
Review essay
Esperti anonimi
Pubblicazione scientifica
2021
MDPI
10
9
1994
1
16
16
Pubblicato
Periodico con rilevanza internazionale
scopus
pubmed
crossref
wos
Aderisco
info:eu-repo/semantics/article
Cardiac imaging in anderson-fabry disease: Past, present and future / R. Esposito, C. Santoro, G.E. Mandoli, V. Cuomo, R. Sorrentino, L. La Mura, M.C. Pastore, F. Bandera, F. D'Ascenzi, A. Malagoli, G. Benfari, A. D'Andrea, M. Cameli. - In: JOURNAL OF CLINICAL MEDICINE. - ISSN 2077-0383. - 10:9(2021), pp. 1994.1-1994.16. [10.3390/jcm10091994]
open
Prodotti della ricerca::01 - Articolo su periodico
13
262
Article (author)
Periodico con Impact Factor
R. Esposito, C. Santoro, G.E. Mandoli, V. Cuomo, R. Sorrentino, L. La Mura, M.C. Pastore, F. Bandera, F. D'Ascenzi, A. Malagoli, G. Benfari, A. D'Andr...espandi
File in questo prodotto:
File Dimensione Formato  
jcm-10-01994.pdf

accesso aperto

Descrizione: Review
Tipologia: Publisher's version/PDF
Dimensione 2.64 MB
Formato Adobe PDF
2.64 MB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/953071
Citazioni
  • ???jsp.display-item.citation.pmc??? 9
  • Scopus 13
  • ???jsp.display-item.citation.isi??? 12
  • OpenAlex ND
social impact