PMID- 30767060
OWN - NLM
STAT- MEDLINE
DCOM- 20200106
LR  - 20210109
IS  - 1861-0692 (Electronic)
IS  - 1861-0684 (Linking)
VI  - 108
IP  - 8
DP  - 2019 Aug
TI  - Non-invasive evaluation of the relationship between electrical and structural 
      cardiac abnormalities in patients with myotonic dystrophy type 1.
PG  - 857-867
LID - 10.1007/s00392-019-01414-0 [doi]
AB  - BACKGROUND: Cardiac involvement in myotonic dystrophy type 1 (MD1) includes 
      conduction disease, arrhythmias, and left-ventricular (LV) systolic dysfunction 
      leading to an increased sudden cardiac death risk. An understanding of the 
      interplay between electrical and structural myocardial changes could improve the 
      prediction of adverse cardiac events. We aimed to explore the relationship 
      between signs of cardiomyopathy by conventional and advanced cardiovascular 
      magnetic resonance (CMR), and electrical abnormalities in MD1. METHODS: 
      Fifty-seven MD1 patients (43 +/- 13 years, 46% male) and 15 matched controls 
      (41 +/- 7 years, 53% male) underwent CMR including cine-imaging with 
      feature-tracking strain analysis, late gadolinium enhancement (LGE), and 
      native/post-contrast T1-mapping with extracellular volume calculation. Standard 
      12-lead and long-term ECG monitoring were performed as screening for rhythm 
      and/or conduction abnormalities. RESULTS: Abnormal ECGs were recorded in 40% of 
      MD1; a pathologic CMR was found in 44%: 21% had an impaired LV-EF and 32% showed 
      non-ischemic LGE. When looking at MD1 patients with available long-term ECG 
      monitoring (n = 39), those with atrial fibrillation (Afib)/flutter(Afl) episodes 
      had lower LV-EF (52 +/- 7 vs. 60 +/- 5%, p = 0.002), lower global longitudinal strain 
      (- 17 +/- 3 vs. - 20 +/- 3%, p = 0.034), a trend to lower left atrial emptying 
      fraction (LA-EF) (44 +/- 14 vs. 55 +/- 8%, p = 0.08), and higher prevalence of LGE 
      (88% vs. 23%, p = 0.001) with an intramural (75% vs. 23%, p = 0.01) and septal 
      (63% vs. 13%, p = 0.009) pattern. In a model including LV-EF (OR 0.8, 95% CI 
      0.7-1.0, p = NS) and LGE presence (OR 14.8, 95% CI 1.4-159.0, p = 0.026), only 
      LGE was independently associated with the occurrence of Afib/Afl episodes. 
      CONCLUSION: Myocardial abnormalities depicted by non-ischemic LGE-CMR were the 
      only independent predictor for the occurrence of Afib/Afl on ECG monitoring, 
      previously shown to predict adverse cardiac events in MD1.
FAU - Chmielewski, Lukas
AU  - Chmielewski L
AD  - Department of Cardiology I, University Hospital Muenster, Munster, Germany.
FAU - Bietenbeck, Michael
AU  - Bietenbeck M
AD  - Department of Cardiology I, University Hospital Muenster, Munster, Germany.
FAU - Patrascu, Alexandru
AU  - Patrascu A
AD  - Division of Cardiology, Robert-Bosch-Hospital, Stuttgart, Germany.
FAU - Rosch, Sabine
AU  - Rosch S
AD  - Division of Cardiology, Robert-Bosch-Hospital, Stuttgart, Germany.
FAU - Sechtem, Udo
AU  - Sechtem U
AD  - Division of Cardiology, Robert-Bosch-Hospital, Stuttgart, Germany.
FAU - Yilmaz, Ali
AU  - Yilmaz A
AD  - Department of Cardiology I, University Hospital Muenster, Munster, Germany.
FAU - Florian, Anca-Rezeda
AU  - Florian AR
AD  - Department of Cardiology I, University Hospital Muenster, Munster, Germany. 
      ancarezeda.florian@ukmuenster.de.
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
DEP - 20190214
PL  - Germany
TA  - Clin Res Cardiol
JT  - Clinical research in cardiology : official journal of the German Cardiac Society
JID - 101264123
SB  - IM
MH  - Adult
MH  - Cardiomyopathies/*diagnosis/physiopathology
MH  - Female
MH  - Follow-Up Studies
MH  - Heart Conduction System/*physiopathology
MH  - Heart Ventricles/*diagnostic imaging/physiopathology
MH  - Humans
MH  - Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Myotonic Dystrophy/*diagnosis/physiopathology
MH  - Retrospective Studies
MH  - Stroke Volume/*physiology
MH  - Ventricular Function, Left/*physiology
OTO - NOTNLM
OT  - Atrial fibrillation
OT  - Atrial flutter
OT  - Cardiovascular magnetic resonance
OT  - Inherited cardiomyopathy
OT  - Myotonic dystrophy
EDAT- 2019/02/16 06:00
MHDA- 2020/01/07 06:00
CRDT- 2019/02/16 06:00
PHST- 2018/10/04 00:00 [received]
PHST- 2019/01/17 00:00 [accepted]
PHST- 2019/02/16 06:00 [pubmed]
PHST- 2020/01/07 06:00 [medline]
PHST- 2019/02/16 06:00 [entrez]
AID - 10.1007/s00392-019-01414-0 [pii]
AID - 10.1007/s00392-019-01414-0 [doi]
PST - ppublish
SO  - Clin Res Cardiol. 2019 Aug;108(8):857-867. doi: 10.1007/s00392-019-01414-0. Epub 
      2019 Feb 14.