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Table 5 Clinical characteristics and imaging findings according to the distribution of late gadolinium enhancement (LGE) and fat infiltration in DM2 and FSHD1 patients at baseline and at follow-up

From: Progressive myocardial injury in myotonic dystrophy type II and facioscapulohumeral muscular dystrophy 1: a cardiovascular magnetic resonance follow-up study

  All DM2 patients DM2 LGE (+) DM2 Fat (+)
Type of Arrhythmias Baseline (n = 32) Follow-up n = 22 Baseline (n = 5) Follow-up n = 6 Baseline n = 6 Follow-up n = 7
SVT (n) 2 9 5 5
Non-sustained VT n (n)
Frequent PVC (≥1000/24h) (n)
AV Block I (n) 4 5 2 2 2 2
AV Block II (n) 1
LBBB 1 1 1 1 1 1
RBBB 1 2 1
LAH 2 3 1
LGE location and global volume    5 6   
Inferolateral basal (n)) 5 6 5 6 1 3
Anterolateral basal (n) 2 2
Septal (n) 1 1 1 1
LGE area - mean (%)    8.4 17.6   
Fat apical      6 7
Medical therapy       
Beta blockers (n) 1 3 1 1
ACE, Sartans (n) 4 10 1 2 2
  All FSHD1 patients FSHD1 LGE (+) FSHD1 Fat (+)
Type of Arrhythmias Baseline (n = 52) Follow-up (n = 40) Baseline (n = 13) Follow-up (n = 15) Baseline (n = 7) Follow-up (n = 12)
SVT (n) 1 8 1 3 5
Non-sustained VT n (n) 1 2 1 2 1 1
Frequent PVC (≥1000/24h) (n) 2 11 1 8 2 3
AV Block I (n) 1 3 1 1 1
AV Block II (n)
LBBB
RBBB
LAH
LGE location and global volume    13 15 2 3
Inferolateral basal (n)) 7 7 7 7
Anterolateral basal (n) 2 1 2 1
Septal (n) 3 3 3 3
Inferior (n) 1 3 1 3
LGE area - mean (%)    18.6 28.9   
Fat apical      7 12
Medical therapy       
Beta blockers (n) 1 4   1 1 3
ACE-I, ARBs (n) 2 7 1 3 1 2
  1. SVT = supraventricular tachycardia, VT = ventricular tachycardia, PVC = premature ventricular contractions, LBBB = left bundle branch block, RBBB = right bundle branch block, LAH = left anterior hemiblock, LGE = late gadolinium enhancement. ACE-I = angiotensin-converting enzyme (ACE) inhibitors, ARBs = angiotensin II receptor blockers