title: Myocardial fibrosis severity on cardiac magnetic resonance imaging predicts sustained arrhythmic events in hypertrophic cardiomyopathy.
abstract: BACKGROUND
  The purpose of our study was to correlate the incidence of adequate implantable cardioverter-defibrillator (ICD) interventions in hypertrophic cardiomyopathy (HCM) patients with risk markers (RMs) for sudden cardiac death (SCD) plus myocardial fibrosis as detected by late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) imaging.
  
  
  METHODS
  In all, 87 patients with HCM underwent LGE-CMR imaging prior to ICD implantation, performed for secondary (n = 2; 2%) or primary SCD prophylaxis (n = 85; 98%). Fibrosis was graded with a 17-segment left ventricular model (0 = absent, 1 = point-shaped, 2 = limited to 1 left ventricular segment, 3 = involving ≥ 2 segments). During follow-up, ICD memories were read out by a physician blinded to the individual patient data.
  
  
  RESULTS
  The number of RMs per patient was 1.9 ± 0.8. Myocardial fibrosis was present in 78 patients (90%); 26 (30%) had a fibrosis score of 3. During follow-up (3.5 ± 2.6 [range, 0.2-11.4 years]), 15 patients had 50 appropriate ICD interventions. Episodes of atrial fibrillation were found in 28 patients. Fibrosis severity correlated with occurrence of ventricular tachycardia (Cramér's V, or φc = 0.4, P < 0.001) and atrial fibrillation (φc = 0.6, P < 0.001). On multivariate regression analysis, an independent association between myocardial fibrosis (ß = 0.6, P < 0.01) and sustained ventricular tachycardia was found.
  
  
  CONCLUSIONS
  In HCM patients treated with ICD implantation because of a high SCD risk by traditional RM assessment, a high rate of arrhythmic events was observed during long-term follow-up. In a cohort of patients with clinical markers for high risk of SCD, severity of myocardial fibrosis as detected by an easy LGE-CMR scoring system was associated with future arrhythmic events and appropriate ICD therapies.
authors: Department of Cardiology, Heart and Diabetes Centre North-Rhine Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany. Cchrprinz@aol.com
authors: Christian
authors: C
authors: Prinz
authors: Maria
authors: M
authors: Schwarz
authors: Ivan
authors: I
authors: Ilic
authors: Kai Thorsten
authors: KT
authors: Laser
authors: Roman
authors: R
authors: Lehmann
authors: Eva-Maria
authors: EM
authors: Prinz
authors: Thomas
authors: T
authors: Bitter
authors: Jürgen
authors: J
authors: Vogt
authors: Frank
authors: F
authors: van Buuren
authors: Nikola
authors: N
authors: Bogunovic
authors: Dieter
authors: D
authors: Horstkotte
authors: Lothar
authors: L
authors: Faber
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D015331:Cohort Studies; D003287:Contrast Media; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable / Q000706:statistics & numerical data; D005260:Female; D005355:Fibrosis; D005500:Follow-Up Studies; D005682:Gadolinium; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009206:Myocardium / Q000473:pathology*; D011237:Predictive Value of Tests; D018570:Risk Assessment; D012307:Risk Factors; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D017180:Tachycardia, Ventricular / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D003287:Contrast Media; D005682:Gadolinium