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Diagnosis and Management of Cardiovascular Involvement in Fabry Disease.
Rubino, Marta; Monda, Emanuele; Lioncino, Michele; Caiazza, Martina; Palmiero, Giuseppe; Dongiglio, Francesca; Fusco, Adelaide; Cirillo, Annapaola; Cesaro, Arturo; Capodicasa, Laura; Mazzella, Marialuisa; Chiosi, Flavia; Orabona, Paolo; Bossone, Eduardo; Calabrò, Paolo; Pisani, Antonio; Germain, Dominique P; Biagini, Elena; Pieroni, Maurizio; Limongelli, Giuseppe.
Affiliation
  • Rubino M; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Monda E; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Lioncino M; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Caiazza M; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Palmiero G; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Dongiglio F; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Fusco A; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Cirillo A; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Cesaro A; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Capodicasa L; Department of Nephrology, Monaldi Hospital, Via L. Bianchi, Naples 80131, Italy.
  • Mazzella M; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Chiosi F; Department of Ophthalmology, Monaldi Hospital, Via L. Bianchi, Naples 80131, Italy.
  • Orabona P; Department of Ophthalmology, Monaldi Hospital, Via L. Bianchi, Naples 80131, Italy.
  • Bossone E; Division of Cardiology, Antonio Cardarelli Hospital, Via A. Cardarelli, Naples 80131, Italy.
  • Calabrò P; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy.
  • Pisani A; Department of Public Health, University Federico II of Naples, Via Pansini, Naples 80131, Italy.
  • Germain DP; French Referral Centre for Fabry Disease, Division of Medical Genetics, Hôpital Raymond-Poincare, AP-HP, Garches 92380, France.
  • Biagini E; Cardiology Unit, St. Orsola Hospital, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, Italy.
  • Pieroni M; Cardiovascular Department, San Donato Hospital, Arezzo, Italy.
  • Limongelli G; Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Via L. Bianchi, Naples 80131, Italy; Institute of Cardiovascular Sciences, University College of London and St. Bartholomew's Hospital, Grower Street, London WC1E 6DD, UK. Electronic address: limongelligiuseppe@
Heart Fail Clin ; 18(1): 39-49, 2022 Jan.
Article in En | MEDLINE | ID: mdl-34776082
Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disease caused by pathogenic variants in the GLA gene. Cardiac involvement is common in FD and is responsible for impaired quality of life and premature death. The classic cardiac involvement is a nonobstructive form of hypertrophic cardiomyopathy, usually manifesting as concentric left ventricular hypertrophy, with subsequent arrhythmogenic intramural fibrosis. Treatment of patients with FD should be directed to prevent the disease progression to irreversible organ damage and organ failure. The aim of this review is to describe the current state of knowledge regarding cardiovascular involvement in FD, focusing on clinical and instrumental features, cardiovascular management, and targeted therapy.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Cardiomyopathy, Hypertrophic / Fabry Disease Type of study: Diagnostic_studies Aspects: Patient_preference Limits: Humans Language: En Journal: Heart Fail Clin Year: 2022 Document type: Article Affiliation country: Italy Country of publication: United States

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Cardiomyopathy, Hypertrophic / Fabry Disease Type of study: Diagnostic_studies Aspects: Patient_preference Limits: Humans Language: En Journal: Heart Fail Clin Year: 2022 Document type: Article Affiliation country: Italy Country of publication: United States