{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T03:53:57Z","timestamp":1790913237263,"version":"4.1.0"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2020,11,5]],"date-time":"2020-11-05T00:00:00Z","timestamp":1604534400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"DM1 patient registry (MYODRAFT study) was funded by the Prinses Beatrix Spierfonds","award":["W.OR15-25"],"award-info":[{"award-number":["W.OR15-25"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,2,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>The aim of this study was to determine electrocardiographic (ECG) criteria predicting abnormal infrahissian conduction in patients with myotonic dystrophy type 1 (DM1), as these criteria could be used to identify the need for an electrophysiological study (EPS).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>A retrospective multicentre study was conducted including DM1-affected individuals who underwent EPS between 2007 and 2018. For each individual, EPS indication, His-ventricle (HV) interval, resting ECG parameters prior to EPS, left ventricular ejection fraction (LVEF), neurological status, and DM1 DNA analysis results were collected. Electrocardiographic parameters of patients with a normal HV interval were compared with ECG parameters of patients with a prolonged HV interval. Logistic regression was performed to determine predictors for a prolonged HV interval of \u226570 ms on EPS and diagnostic accuracy of ECG parameters was ascertained. Among 100 DM1-affected individuals undergoing EPS, 47 had a prolonged HV interval. The sole presence of a PR interval &amp;gt;200 ms [odds ratio (OR) 8.45, confidence interval (CI) 2.64\u201327.04] or a QRS complex &amp;gt;120 ms (OR 9.91, CI 3.53\u201327.80) on ECG were independent predictors of a prolonged HV interval. The combination of both parameters had a positive predictive value of 78% for delayed infrahissian conduction on EPS. His-ventricle interval was independent of DM1 genetic mutation size, neuromuscular status, and LVEF.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>The combination of a prolonged PR interval and widened QRS complex on ECG accurately predicts abnormal infrahissian conduction on EPS in patients with DM1. These ECG parameters could be used as a screening tool to determine the need for referral to a specialized multidisciplinary neuromuscular team with EPS capacity.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/europace\/euaa256","type":"journal-article","created":{"date-parts":[[2020,8,4]],"date-time":"2020-08-04T19:28:17Z","timestamp":1596569297000},"page":"298-304","source":"Crossref","is-referenced-by-count":20,"title":["Electrocardiographic predictors of infrahissian conduction disturbances in myotonic dystrophy type 1"],"prefix":"10.1093","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1110-6172","authenticated-orcid":false,"given":"Isis B T","family":"Joosten","sequence":"first","affiliation":[{"name":"Department of Neurology, Maastricht University Medical Center+, PO Box 5800, 6202 AZ Maastricht, The Netherlands"},{"name":"School for Mental Health and Neuroscience, Maastricht University, Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romy","family":"van Lohuizen","sequence":"additional","affiliation":[{"name":"Department of Neurology, Maastricht University Medical Center+, PO Box 5800, 6202 AZ Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis W","family":"den Uijl","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Medical Center+, Maastricht, The Netherlands"},{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinder","family":"Evertz","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Medical Center+, Maastricht, The Netherlands"},{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bianca T A","family":"de Greef","sequence":"additional","affiliation":[{"name":"Department of Neurology, Maastricht University Medical Center+, PO Box 5800, 6202 AZ Maastricht, The Netherlands"},{"name":"Department of Clinical Epidemiology and Medical Technology Assessment, Maastricht University Medical Center+, Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baziel G M","family":"van Engelen","sequence":"additional","affiliation":[{"name":"Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2467-067X","authenticated-orcid":false,"given":"Catharina G","family":"Faber","sequence":"additional","affiliation":[{"name":"Department of Neurology, Maastricht University Medical Center+, PO Box 5800, 6202 AZ Maastricht, The Netherlands"},{"name":"School for Mental Health and Neuroscience, Maastricht University, Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Vernooy","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Medical Center+, Maastricht, The Netherlands"},{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,11,5]]},"reference":[{"key":"2021020808560001000_euaa256-B1","doi-asserted-by":"crossref","first-page":"799","DOI":"10.1016\/0092-8674(92)90154-5","article-title":"Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member","volume":"68","author":"Brook","year":"1992","journal-title":"Cell"},{"key":"2021020808560001000_euaa256-B2","doi-asserted-by":"crossref","first-page":"1557","DOI":"10.1093\/brain\/121.8.1557","article-title":"Age and causes of death in adult-onset myotonic dystrophy","volume":"121","author":"de Die-Smulders","year":"1998","journal-title":"Brain"},{"key":"2021020808560001000_euaa256-B3","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1136\/heart.88.6.665","article-title":"Myotonic dystrophy and the heart","volume":"88","author":"Pelargonio","year":"2002","journal-title":"Heart"},{"key":"2021020808560001000_euaa256-B4","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1016\/j.ijcard.2011.08.037","article-title":"Cardiac manifestations of myotonic dystrophy type 1","volume":"160","author":"Petri","year":"2012","journal-title":"Int J Cardiol"},{"key":"2021020808560001000_euaa256-B5","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1136\/heartjnl-2011-300143","article-title":"The evolution of infrahissian conduction time in myotonic dystrophy patients: clinical implications","volume":"98","author":"Lallemand","year":"2012","journal-title":"Heart"},{"key":"2021020808560001000_euaa256-B6","doi-asserted-by":"crossref","first-page":"1645","DOI":"10.1016\/S0735-1097(02)02339-2","article-title":"Long-term follow-up of arrhythmias in patients with myotonic dystrophy treated by pacing: a multicenter diagnostic pacemaker study","volume":"40","author":"Lazarus","year":"2002","journal-title":"J Am Coll Cardiol"},{"key":"2021020808560001000_euaa256-B7","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/j.ijcard.2010.02.029","article-title":"Mortality in myotonic dystrophy patients in the area of prophylactic pacing devices","volume":"150","author":"Laurent","year":"2011","journal-title":"Int J Cardiol"},{"key":"2021020808560001000_euaa256-B8","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1016\/j.jacc.2018.10.043","article-title":"ACC\/AHA\/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: executive Summary: a Report of the American College of Cardiology\/American Heart Association Task Force on Clinical Practice Guidelines, and the Heart Rhythm Society","volume":"74","author":"Kusumoto","year":"2019","journal-title":"J Am Coll Cardiol"},{"key":"2021020808560001000_euaa256-B9","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1093\/europace\/eut206","article-title":"2013 ESC guidelines on cardiac pacing and cardiac resynchronization therapy: the task force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA)","volume":"15","author":"Brignole","year":"2013","journal-title":"Europace"},{"key":"2021020808560001000_euaa256-B10","doi-asserted-by":"crossref","first-page":"2688","DOI":"10.1056\/NEJMoa062800","article-title":"Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1","volume":"358","author":"Groh","year":"2008","journal-title":"N Engl J Med"},{"key":"2021020808560001000_euaa256-B11","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1212\/WNL.56.3.336","article-title":"Assessment of a disease-specific muscular impairment rating scale in myotonic dystrophy","volume":"56","author":"Mathieu","year":"2001","journal-title":"Neurology"},{"key":"2021020808560001000_euaa256-B12","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1136\/hrt.82.5.634","article-title":"Is it possible to identify infrahissian cardiac conduction abnormalities in myotonic dystrophy by non-invasive methods?","volume":"82","author":"Babuty","year":"1999","journal-title":"Heart"},{"key":"2021020808560001000_euaa256-B13","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1001\/jama.2012.346","article-title":"Electrophysiological study with prophylactic pacing and survival in adults with myotonic dystrophy and conduction system disease","volume":"307","author":"Wahbi","year":"2012","journal-title":"JAMA"},{"key":"2021020808560001000_euaa256-B14","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1016\/j.ijcard.2014.02.001","article-title":"Insufficiency of electrocardiogram alone in predicting infrahisian abnormalities in patients with type 1 myotonic dystrophy","volume":"172","author":"Simeon","year":"2014","journal-title":"Int J Cardiol"},{"key":"2021020808560001000_euaa256-B15","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1161\/01.CIR.99.8.1041","article-title":"Relationships among electrophysiological findings and clinical status, heart function, and extent of DNA mutation in myotonic dystrophy","volume":"99","author":"Lazarus","year":"1999","journal-title":"Circulation"},{"key":"2021020808560001000_euaa256-B16","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1093\/europace\/euq423","article-title":"Long-term follow-up of patients with myotonic dystrophy: an electrocardiogram every year is not necessary","volume":"13","author":"Brembilla-Perrot","year":"2011","journal-title":"Europace"},{"key":"2021020808560001000_euaa256-B17","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1161\/01.CIR.60.6.1360","article-title":"The natural history of conduction system disease in myotonic muscular dystrophy as determined by serial electrophysiologic studies","volume":"60","author":"Prystowsky","year":"1979","journal-title":"Circulation"},{"key":"2021020808560001000_euaa256-B18","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1046\/j.1540-8167.2002.00444.x","article-title":"Severity of cardiac conduction involvement and arrhythmias in myotonic dystrophy type 1 correlates with age and CTG repeat length","volume":"13","author":"Groh","year":"2002","journal-title":"J Cardiovasc Electrophysiol"},{"key":"2021020808560001000_euaa256-B19","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1016\/j.bbadis.2014.05.019","article-title":"Myotonic dystrophies: an update on clinical aspects, genetic, pathology, and molecular pathomechanisms","volume":"1852","author":"Meola","year":"2015","journal-title":"Biochim Biophys Acta"},{"key":"2021020808560001000_euaa256-B20","doi-asserted-by":"crossref","first-page":"99","DOI":"10.3233\/JND-140025","article-title":"DM1 patients with small CTG expansions are also at risk of severe conduction abnormalities","volume":"2","author":"Denicourt","year":"2015","journal-title":"J Neuromuscular Diseases"}],"container-title":["EP Europace"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/europace\/article-pdf\/23\/2\/298\/36209372\/euaa256.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/europace\/article-pdf\/23\/2\/298\/36209372\/euaa256.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,2,8]],"date-time":"2021-02-08T19:37:42Z","timestamp":1612813062000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/europace\/article\/23\/2\/298\/5956388"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,11,5]]},"references-count":20,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2020,11,5]]},"published-print":{"date-parts":[[2021,2,5]]}},"URL":"https:\/\/doi.org\/10.1093\/europace\/euaa256","relation":{},"ISSN":["1099-5129","1532-2092"],"issn-type":[{"value":"1099-5129","type":"print"},{"value":"1532-2092","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,2,1]]},"published":{"date-parts":[[2020,11,5]]}}}