title: Myocardial fibrosis in patients with myotonic dystrophy type 1: a cardiovascular magnetic resonance study.
abstract: BACKGROUND
  Myotonic dystrophy type 1 (DM1) is associated with increased cardiac morbidity and mortality. Therefore, assessment of cardiac involvement and risk stratification for sudden cardiac death is crucial. Nevertheless, optimal screening-procedures are not clearly defined. ECG, echocardiography and Holter-monitoring are useful but insufficient. Cardiovascular magnetic resonance (CMR) can provide additional information of which myocardial fibrosis may be relevant. The purpose of this study was to describe the prevalence of myocardial fibrosis in patients with DM1 assessed by CMR, and the association between myocardial fibrosis and abnormal findings on ECG, Holter-monitoring and echocardiography.
  
  
  METHODS
  We selected 30 unrelated patients with DM1: 18 patients (10 men, mean age 51 years) with, and 12 patients (7 men, mean age 41 years) without abnormal findings on ECG and Holter-monitoring. Patients were evaluated with medical history, physical examination, ECG, Holter-monitoring, echocardiography and CMR.
  
  
  RESULTS
  Myocardial fibrosis was found in 12/30 (40%, 9 men). The presence of myocardial fibrosis was associated with the following CMR-parameters: increased left ventricular mass (median (range) 55 g/m² (43-83) vs. 46 g/m² (36-64), p = 0.02), increased left atrial volume (median (range) 52 ml/m² (36-87) vs. 46 ml/m² (35-69), p = 0.04) and a trend toward lower LVEF (median (range) 63% (38-71) vs. 66% (60-80), p = 0.06). Overall, we found no association between the presence of myocardial fibrosis and abnormal findings on: ECG (p = 0.71), Holter-monitoring (p = 0.27) or echocardiographic measurements of left ventricular volumes, ejection fraction or global longitudinal strain (p = 0.18).
  
  
  CONCLUSIONS
  Patients with DM1 had a high prevalence of myocardial fibrosis which was not predicted by ECG, Holter-monitoring or echocardiography. CMR add additional information to current standard cardiac assessment and may prove to be a clinically valuable tool for risk stratification in DM1.
authors.affiliation: Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark. Hellepetri1@gmail.com.
authors.forename: Helle
authors.initials: H
authors.lastname: Petri
authors.affiliation: Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark. kirilaleksov@live.dk.
authors.forename: Kiril Aleksov
authors.initials: KA
authors.lastname: Ahtarovski
authors.affiliation: Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark. niels.vejlstrup@regionh.dk.
authors.forename: Niels
authors.initials: N
authors.lastname: Vejlstrup
authors.affiliation: Neuromuscular Research Unit, Department of Neurology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. john.vissing@regionh.dk.
authors.forename: John
authors.initials: J
authors.lastname: Vissing
authors.affiliation: Neuromuscular Research Unit, Department of Neurology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. nanna.witting@regionh.dk.
authors.forename: Nanna
authors.initials: N
authors.lastname: Witting
authors.affiliation: Department of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark. lk@heart.dk.
authors.forename: Lars
authors.initials: L
authors.lastname: Køber
authors.affiliation: Unit for Inherited Cardiac Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark. henningbundgaard@dadlnet.dk.
authors.forename: Henning
authors.initials: H
authors.lastname: Bundgaard
mesh_terms: D000328:Adult; D000368:Aged; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000473:pathology / Q000503:physiopathology; D016022:Case-Control Studies; D003718:Denmark / Q000453:epidemiology; D015716:Electrocardiography, Ambulatory; D005260:Female; D005355:Fibrosis; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction; D009206:Myocardium / Q000473:pathology*; D009223:Myotonic Dystrophy / Q000453:epidemiology*; D011237:Predictive Value of Tests; D015995:Prevalence; D013318:Stroke Volume; D014463:Ultrasonography; D016277:Ventricular Function, Left
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