{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T03:27:15Z","timestamp":1793935635921,"version":"4.8.0"},"reference-count":312,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2026,1,18]],"date-time":"2026-01-18T00:00:00Z","timestamp":1768694400000},"content-version":"vor","delay-in-days":2027,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2020,8,14]],"date-time":"2020-08-14T00:00:00Z","timestamp":1597363200000},"content-version":"vor","delay-in-days":44,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Fabry disease (FD) is an X-linked lysosomal storage disorder caused by pathogenic variants in the \u03b1-galactosidase A (GLA) gene that leads to reduced or undetectable \u03b1-galactosidase A enzyme activity and progressive accumulation of globotriaosylceramide and its deacylated form globotriaosylsphingosine in cells throughout the body. FD can be multisystemic with neurological, renal, cutaneous and cardiac involvement or be limited to the heart. Cardiac involvement is characterized by progressive cardiac hypertrophy, fibrosis, arrhythmias, heart failure and sudden cardiac death. The cardiac management of FD requires specific measures including enzyme replacement therapy or small pharmacological chaperones in patients carrying amenable pathogenic GLA gene variants and more general management of cardiac symptoms and complications. In this paper, we summarize current knowledge of FD-related heart disease and expert consensus recommendations for its management.<\/jats:p>","DOI":"10.1002\/ejhf.1960","type":"journal-article","created":{"date-parts":[[2020,7,8]],"date-time":"2020-07-08T12:09:01Z","timestamp":1594210141000},"page":"1076-1096","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":215,"title":["An Expert Consensus Document on the Management of Cardiovascular Manifestations of Fabry Disease"],"prefix":"10.1093","volume":"22","author":[{"given":"Ale\u0161","family":"Linhart","sequence":"first","affiliation":[{"name":"Second Department of Internal Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital in Prague , Prague ,","place":["Czech Republic"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique P.","family":"Germain","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, University of Versailles and AP-HP Paris-Saclay , Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iacopo","family":"Olivotto","sequence":"additional","affiliation":[{"name":"Cardiomyopathy Unit, Careggi University Hospital , Florence ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed M.","family":"Akhtar","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London and Barts Heart Centre , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aris","family":"Anastasakis","sequence":"additional","affiliation":[{"name":"Unit of Inherited and Rare Cardiovascular Diseases, Onassis Cardiac Surgery Center , Kallithea ,","place":["Greece"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derralynn","family":"Hughes","sequence":"additional","affiliation":[{"name":"Royal Free London NHS Foundation Trust and University College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehdi","family":"Namdar","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Specialties, Cardiology, Electrophysiology, University Hospital of Geneva , Geneva ,","place":["Switzerland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Pieroni","sequence":"additional","affiliation":[{"name":"Cardiomyopathy Clinic, Cardiovascular Department, San Donato Hospital , Arezzo ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert","family":"Hag\u00e8ge","sequence":"additional","affiliation":[{"name":"Cardiology Department, Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pital Europ\u00e9en Georges Pompidou , Paris ,","place":["France"]},{"name":"Universit\u00e9 Paris Descartes, Sorbonne Paris Cit\u00e9 , Paris ,","place":["France"]},{"name":"INSERM CMR970, Paris Cardiovascular Research Center PARCC , Paris ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franco","family":"Cecchi","sequence":"additional","affiliation":[{"name":"Cardiomyopathy Unit, Careggi University Hospital , Florence ,","place":["Italy"]},{"name":"IRCCS, Istituto Auxologico Italiano, Department of Cardiovascular, Neural and Metabolic Sciences, San Luca Hospital , Milan ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan R.","family":"Gimeno","sequence":"additional","affiliation":[{"name":"Hospital C. 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