{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T03:54:32Z","timestamp":1790913272920,"version":"4.1.0"},"reference-count":14,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"17","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2020,9]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              This study enrolled 506 patients with myotonic dystrophy type 1 (aged \u226515\u00a0years; &gt;50 cytosine\u2010thymine\u2010guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow\u2010up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow\u2010up period of 87 months (Q1\u2013Q3, 37\u2013138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22\u201315.50) were associated with sudden death. In contrast, PQ interval \u2265240\u00a0ms, QRS duration \u2265120\u00a0ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval \u2265240\u00a0ms (HR, 2.79; 95% CI, 1.9\u20137.19,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.05) or QRS duration \u2265120\u00a0ms (HR, 9.41; 95% CI, 2.62\u201333.77,\n              <jats:italic>P<\/jats:italic>\n              &lt; 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval &lt;240\u00a0ms or QRS duration &lt;120\u00a0ms; these cardiac conduction parameters were not related to sudden death.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.119.015709","type":"journal-article","created":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T09:03:49Z","timestamp":1597827829000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":19,"title":["Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1"],"prefix":"10.1161","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5104-3313","authenticated-orcid":false,"given":"Hideki","family":"Itoh","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine Shiga University of Medical Science Otsu Japan"},{"name":"Division of Patient Safety Hiroshima University Hospital Hiroshima Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Hisamatsu","sequence":"additional","affiliation":[{"name":"Department of Public Health Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Okayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takuhisa","family":"Tamura","sequence":"additional","affiliation":[{"name":"Department of Neurology National Hospital Organization Higashisaitama National Hospital Saitama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuhiko","family":"Segawa","sequence":"additional","affiliation":[{"name":"Department of Cardiology National Center Hospital National Center of Neurology and Psychiatry Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiaki","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Neurology National Hospital Organization Sendai\u2010Nishitaga Hospital 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Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shugo","family":"Suwazono","sequence":"additional","affiliation":[{"name":"Division of Neurology and Center for Clinical Neuroscience National Hospital Organization Okinawa National Hospital Ginowan Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shingo","family":"Sasaki","sequence":"additional","affiliation":[{"name":"Department of Advanced Management of Cardiac Arrhythmias Hirosaki University Graduate School of Medicine Hirosaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken","family":"Okumura","sequence":"additional","affiliation":[{"name":"Advanced Arrhythmia Therapeutic Branch Division of Cardiology Saiseikai Kumamoto Hospital Cardiovascular Center Kumamoto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minoru","family":"Horie","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine Shiga University of Medical Science Otsu Japan"},{"name":"Center for Epidemiologic Research in Asia and Department of Cardiology Shiga University of Medical Science Otsu Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masanori P.","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Functional Diagnostic Science Osaka University Graduate School of Medicine Suita Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsuyoshi","family":"Matumura","sequence":"additional","affiliation":[{"name":"Department of Neurology National Hospital Organization Osaka Toneyama Medical Center 5\u20101\u20101 Toyonaka Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa062800"},{"key":"e_1_3_1_3_2","doi-asserted-by":"crossref","first-page":"e014004","DOI":"10.1161\/JAHA.119.014004","article-title":"Cardiac intervention improves heart disease and 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