{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T16:54:33Z","timestamp":1791305673585,"version":"4.3.1"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T00:00:00Z","timestamp":1790035200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>The IVS4+919G&amp;gt;A variant is the most prevalent late-onset Fabry variant in East Asian populations, but its electrophysiological trajectory remains incompletely defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>From a regional genetic screening programme, 905 IVS4+919G&amp;gt;A carriers were identified among 457 581 patients and matched 1:2 to controls by age and sex. Electrocardiographic (ECG) parameters were compared across decades, and echocardiography correlated structural changes with ECG findings. The cumulative prevalence of IVS4+919G&amp;gt;A was 0.198%. Age-specific prevalence in females was \u223c2.5-fold higher than in males (0.11\u20130.15%), without significant change throughout different age categories. Female carriers demonstrated the catch-up phenomenon after the age of 60. Males exhibited a nine-fold risk of right bundle branch block and pacemaker implantation compared with male controls (right bundle branch block, HR 9.286, 95% CI 5.16\u201316.70, P &amp;lt; 0.001; pacemaker implantation, HR 9.197, 95% CI 2.88\u201329.31, P &amp;lt; 0.001), and this precedes the onset of echocardiographic left ventricular hypertrophy. Risk of stroke and atrial fibrillation (HR 1.435, 95% CI 0.69\u20132.96, P = 0.289) was similar between male carriers and controls. PR interval did not shorten but was prolonged instead. Combining left ventricular hypertrophy with conduction abnormalities, ECG detection achieved 88% sensitivity, exceeding echocardiographic criteria alone (51.5%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The IVS4+919G&amp;gt;A variant is characterized by a progressive electrical phenotype dominated by conduction system disease preceding hypertrophy without excess major arrhythmic events. Electrocardiographic surveillance and phenotype-based risk stratification may be more informative than structural assessment alone for identifying individuals at risk of device therapy.<\/jats:p>\n                    <jats:p>Institutional Review Board: CMUH113-REC3-076<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/europace\/euag258","type":"journal-article","created":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T11:33:17Z","timestamp":1790076797000},"source":"Crossref","is-referenced-by-count":0,"title":["Progressive conduction system disease preceding hypertrophy in IVS4+919G&amp;gt;A Fabry variant: a population-based electrocardiographic study"],"prefix":"10.1093","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8451-4011","authenticated-orcid":false,"given":"Wei-Hsin","family":"Chung","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine, Taichung Municipal Geriatric Rehabilitation General Hospital , Taichung ,","place":["Taiwan"]},{"name":"Division of Cardiology, Department of Medicine, China Medical University Hospital , Taichung ,","place":["Taiwan"]},{"name":"Metal Industries Research and Development Centre , Kaohsiung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-5529-3213","authenticated-orcid":false,"given":"Cheng-Chang","family":"Tung","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Taichung Municipal Geriatric Rehabilitation General Hospital , Taichung ,","place":["Taiwan"]},{"name":"Division of Cardiology, Department of Medicine, China Medical University Hospital , Taichung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting-Yuan","family":"Liu","sequence":"additional","affiliation":[{"name":"Million-person Precision Medicine Initiative, Department of Medical Research, China Medical University Hospital , Taichung ,","place":["Taiwan"]},{"name":"Master Program for Digital Health Innovation, China Medical University , Taichung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wan-Jung","family":"Chang","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, China Medical University Hospital , Taichung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6476-9896","authenticated-orcid":false,"given":"Kuan-Cheng","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Taichung Municipal Geriatric Rehabilitation General Hospital , Taichung ,","place":["Taiwan"]},{"name":"Division of Cardiology, Department of Medicine, China Medical University Hospital , Taichung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei-De","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Medical Research, China Medical University Hospital , Taichung 404332 ,","place":["Taiwan"]},{"name":"Genetic Center, Department of Medical Research, China Medical University Hospital , Taichung ,","place":["Taiwan"]},{"name":"School of Post Baccalaureate Chinese Medicine, China Medical University , Taichung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3469-7443","authenticated-orcid":false,"given":"Hsin-Yueh","family":"Liang","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, China Medical University Hospital , Taichung ,","place":["Taiwan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1373-245X","authenticated-orcid":false,"given":"Fuu-Jen","family":"Tsai","sequence":"additional","affiliation":[{"name":"Department of Medical Research, China Medical University Hospital , Taichung 404332 ,","place":["Taiwan"]},{"name":"School of Chinese Medicine, China Medical University , No. 100, Sec. 1, Jingmao Rd., Beitun Dist., Taichung 406040 ,","place":["Taiwan"]},{"name":"Division of Medical Genetics, Children\u2019s Hospital of China Medical University , No. 95, Xueshi Rd., North Dist., Taichung 404332 ,","place":["Taiwan"]},{"name":"Department of Medical Laboratory Science and Biotechnology, Asia University , No. 500, Lioufeng Rd., Wufeng Dist., Taichung 413054 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