{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T01:13:37Z","timestamp":1782782017444,"version":"3.54.5"},"reference-count":31,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,5,17]],"date-time":"2021-05-17T00:00:00Z","timestamp":1621209600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100013995","name":"Sanofi Genzyme","doi-asserted-by":"publisher","award":["GZ-2015-11485"],"award-info":[{"award-number":["GZ-2015-11485"]}],"id":[{"id":"10.13039\/100013995","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Hong Kong Special Administrative Region Government Health and Medical Research Fund","award":["05160976"],"award-info":[{"award-number":["05160976"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Left ventricular hypertrophy (LVH) caused by cardiac variant Fabry disease (FD) is typically late-onset and may mimic LVH caused by abnormal loading conditions. We aimed to determine the prevalence of FD in a non-selective patient population of everyday practice presenting with LVH, including those with hypertension and valve disease. We measured plasma alpha-galactosidase A activity using dried blood spot tests in 499 (age = 66 \u00b1 13 years; 336 men) Hong Kong Chinese patients with LVH defined as maximal LV septal\/posterior wall thickness \u226513 mm on echocardiography. Patients with low enzyme activity underwent mutation analysis of the GLA gene. Eight (age = 53\u221274 years; all men) unrelated patients (1.6%) had low plasma alpha-galactosidase A activity (0.57 \u00b1 0.27 \u03bcmol\/L wb\/hr) and all were confirmed to have the GLA IVS4 + 919G &gt; A mutation. FD patients presented with heart failure (n = 5), heart block (n = 2), ventricular tachycardia (n = 1), chest pain (n = 3), and\/or murmur (n = 1). Uncontrolled hypertension (n = 4) and\/or severe mitral\/aortic valve pathology (n = 2) were frequent. Ethnic subgroups included Teochew (n = 5), Canton (n = 2), and Wenzhou (n = 1). Endomyocardial biopsy (n = 6) revealed hypertrophic myocytes with vacuolization and dense lamellar bodies. Late-onset IVS4 + 919G &gt; A FD is prevalent among Chinese LVH patients, and should be considered as a cause of LVH in adult patients even when hypertension and\/or valve pathology are present.<\/jats:p>","DOI":"10.3390\/jcm10102160","type":"journal-article","created":{"date-parts":[[2021,5,17]],"date-time":"2021-05-17T05:45:13Z","timestamp":1621230313000},"page":"2160","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["High Prevalence of Late-Onset Fabry Cardiomyopathy in a Cohort of 499 Non-Selective Patients with Left Ventricular Hypertrophy: The Asian Fabry Cardiomyopathy High-Risk Screening Study (ASIAN-FAME)"],"prefix":"10.3390","volume":"10","author":[{"given":"Yiting","family":"Fan","sequence":"first","affiliation":[{"name":"Cardiology Department, Shanghai Chest Hospital, Shanghai Jiaotong University, Shanghai 200030, China"},{"name":"Laboratory of Cardiac Imaging and 3D Printing, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0361-6903","authenticated-orcid":false,"given":"Tsz-Ngai","family":"Chan","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiac Imaging and 3D Printing, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong, China"},{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1588-2072","authenticated-orcid":false,"given":"Josie T. Y.","family":"Chow","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiac Imaging and 3D Printing, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong, China"},{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin K. H.","family":"Kam","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4296-3035","authenticated-orcid":false,"given":"Wai-Kin","family":"Chi","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph Y. S.","family":"Chan","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erik","family":"Fung","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mabel M. P.","family":"Tong","sequence":"additional","affiliation":[{"name":"Department of Radiology, Alice Ho Miu Ling Nethersole Hospital, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffery K. T.","family":"Wong","sequence":"additional","affiliation":[{"name":"Department of Imaging and Interventional Radiology, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul C. L.","family":"Choi","sequence":"additional","affiliation":[{"name":"Department of Anatomical and Cellular Pathology, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David K. H.","family":"Chan","sequence":"additional","affiliation":[{"name":"Clinical Genetic Service, Department of Health, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bun","family":"Sheng","sequence":"additional","affiliation":[{"name":"Princess Margaret Hospital, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4120-155X","authenticated-orcid":false,"given":"Alex Pui-Wai","family":"Lee","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiac Imaging and 3D Printing, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong, China"},{"name":"Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Hong Kong, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,17]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1016\/j.acvd.2019.01.002","article-title":"Fabry disease in cardiology practice: Literature review and expert point of view","volume":"112","author":"Hagege","year":"2019","journal-title":"Arch. 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