{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T19:16:23Z","timestamp":1794078983980,"version":"4.8.0"},"reference-count":26,"publisher":"American Medical Association (AMA)","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Cardiol"],"published-print":{"date-parts":[[2025,11,1]]},"abstract":"<jats:sec id=\"ab-hoi250048-4\">\n                    <jats:title>Importance<\/jats:title>\n                    <jats:p>\n                      For the prevention of sudden cardiac death in myotonic dystrophy type 1 (\n                      <jats:italic>dystrophia myotonica<\/jats:italic>\n                      ; DM1), professional practice guidelines recommend pacemaker implantation in asymptomatic patients with a PR interval greater than or equal to 240 milliseconds and\/or QRS duration greater than or equal to 120 milliseconds on electrocardiogram (ECG), or a His-ventricular (HV) interval greater than or equal to 70 milliseconds during electrophysiological study (EPS), as class IIa indications.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-hoi250048-5\">\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To determine which of these strategies\u2014ECG or EPS based\u2014is more effective in predicting major bradyarrhythmic events (MBAEs).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-hoi250048-6\">\n                    <jats:title>Design, Setting, and Participants<\/jats:title>\n                    <jats:p>This was a cohort analysis of retrospectively collected longitudinal data from the DM1 Heart Registry. The setting included cardiology and neurology departments of 6 French university hospitals. Study participants were selected from individuals enrolled in the DM1 Heart Registry between 2000 and 2020. The DM1 Heart Registry includes adults with genetically confirmed DM1. Included patients had a history of first EPS after 1999 and no personal history of advanced atrioventricular block or sustained ventricular tachycardia. Study data were analyzed from January to July 2025.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-hoi250048-7\">\n                    <jats:title>Exposures<\/jats:title>\n                    <jats:p>ECG- and EPS-based strategies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-hoi250048-8\">\n                    <jats:title>Main Outcomes and Measures<\/jats:title>\n                    <jats:p>The primary outcome was MBAEs, defined as sudden cardiac death, resuscitated cardiac arrest, or second-degree type II or complete atrioventricular block.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-hoi250048-9\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 1778 adults with genetically confirmed DM1 enrolled in the DM1 Heart Registry, a total of 706 patients (mean [SD] age, 42\u2009[13] years; 359 male [51%]) were included in this study. At baseline, 273 patients (38%) had an HV interval greater than or equal to 70 milliseconds, and 232 (32%) met ECG criteria. Over a median (IQR) follow-up of 5.9 (2.3-9.7) years, 99 patients (14%) experienced an MBAE. In multivariable Cox and joint models incorporating baseline and time-varying values of PR and QRS durations, the HV interval was the only variable significantly associated with the incidence of MBAEs (hazard ratio [HR],\u20091.77; 95% CI, 1.46-2.16;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&amp;amp;lt;\u2009.001 and HR,\u20091.78; 95% CI, 1.40-2.22;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u2009.001, respectively). Compared with ECG-based criteria, the EPS criterion proved to be a more reliable (HR, 2.89; 95% CI, 1.92-4.34 vs HR, 1.95; 95% CI, 1.31-2.89) and more sensitive (performance index [SE], 68.35% [6.24%] vs 34.76% [6.47%]) predictor of MBAE and accurately reclassified 28.8% of patients with an MBAE. Lowering the threshold to HV greater than or equal to 65 milliseconds further improved sensitivity (performance index [SE], 90.18% [3.85%]) and net reclassification improvement (33.7%; 95% CI, 19.6%-48.2%) for MBAE prediction.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec id=\"ab-hoi250048-10\">\n                    <jats:title>Conclusions and Relevance<\/jats:title>\n                    <jats:p>In this cohort of patients with DM1, the HV interval outperformed ECG criteria in predicting MBAEs. An HV threshold greater than or equal to 65 milliseconds may enhance risk stratification for prophylactic pacing.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1001\/jamacardio.2025.3055","type":"journal-article","created":{"date-parts":[[2025,9,24]],"date-time":"2025-09-24T15:31:14Z","timestamp":1758727874000},"page":"1121","source":"Crossref","is-referenced-by-count":12,"title":["Electrocardiogram vs Electrophysiological Study and Major Conduction Delays in Myotonic Dystrophy Type 1"],"prefix":"10.1001","volume":"10","author":[{"given":"Nicolas","family":"Clementy","sequence":"first","affiliation":[{"name":"Department of Cardiology, Universit\u00e9 de Tours, CHU de Tours, Paris, France"},{"name":"Clinique du Mill\u00e9naire, Montpellier, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabien","family":"Labombarda","sequence":"additional","affiliation":[{"name":"University Hospital of Caen, Caen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Grolleau","sequence":"additional","affiliation":[{"name":"INSERM U1153, Paris, France"},{"name":"Universit\u00e9 Paris Descartes - Sorbonne Paris Cit\u00e9, Paris, France"},{"name":"Centre d\u2019Epid\u00e9miologie Clinique, H\u00f4pital H\u00f4tel-Dieu, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Algalarrondo","sequence":"additional","affiliation":[{"name":"Service de Cardiologie, H\u00f4pital Bichat-Claude-Bernard, AP-HP, Paris, France"},{"name":"Universit\u00e9 de Paris, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Bassez","sequence":"additional","affiliation":[{"name":"Centre de Recherche en Myologie-UMR 974, Service de Neuro-Myologie, Institut de Myologie, H\u00f4pital Universitaire Piti\u00e9-Salp\u00eatri\u00e8re, Sorbonne-Universit\u00e9, INSERM, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henri-Marc","family":"B\u00e9cane","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence des Maladies Neuromusculaires Nord\/Est\/Ile-de-France, Institut de Myologie, Service de Neuromyologie, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"B\u00e9hin","sequence":"additional","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence des Maladies Neuromusculaires Nord\/Est\/Ile-de-France, Institut de Myologie, Service de Neuromyologie, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, APHP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7oise","family":"Chapon","sequence":"additional","affiliation":[{"name":"Centre de Comp\u00e9tences des Pathologies Neuromusculaires, CHU de Caen, Caen, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"El Hachmi","sequence":"additional","affiliation":[{"name":"Department of Genetics, European Georges Pompidou Hospital, APHP, Paris, France"},{"name":"Department of Molecular Medicine, Sapienza University of Rome, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdallah","family":"Fayssoil","sequence":"additional","affiliation":[{"name":"University of Paris-Saclay, INSERM U1179, END-ICAP, Versailles, France"},{"name":"Department of Cardiology, Ambroise Par\u00e9 Hospital, APHP, UVSQ, INSERM U1018, Centre de R\u00e9f\u00e9rence des Cardiomyopathies, Boulogne Billancourt, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bertrand","family":"Fontaine","sequence":"additional","affiliation":[{"name":"Centre de Recherche en Myologie-UMR 974, Service de Neuro-Myologie, Institut de Myologie, H\u00f4pital Universitaire Piti\u00e9-Salp\u00eatri\u00e8re, Sorbonne-Universit\u00e9, INSERM, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rodrigue","family":"Garcia","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Hospital of Poitiers, Poitiers, France"},{"name":"Centre d\u2019Investigation Clinique CIC1402, CHU Poitiers, Poitiers, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Lafor\u00eat","sequence":"additional","affiliation":[{"name":"Nord\/Est\/\u00cele-de-France Neuromuscular Reference Center, Department of Neurology, Raymond-Poincar\u00e9 Teaching Hospital, AP-HP, Garches, France"},{"name":"INSERM U1179, END-ICAP, Versailles-Saint-Quentin-en-Yvelines University, Universit\u00e9 Paris Saclay, Montigny-le-Bretonneux, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnaud","family":"Lazarus","sequence":"additional","affiliation":[{"name":"GHP A. 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