title: Role of late gadolinium enhancement cardiovascular magnetic resonance in the risk stratification of hypertrophic cardiomyopathy.
abstract: OBJECTIVE
  Myocardial fibrosis identified by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM) is associated with adverse cardiovascular events, but its value as an independent risk factor for sudden cardiac death (SCD) is unknown. We investigated the role of LGE-CMR in the risk stratification of HCM.
  
  
  METHODS
  We conducted a prospective cohort study in a tertiary referral centre. Consecutive patients with HCM (n=711, median age 56.3 years, IQR 46.7-66.6; 70.0% male) underwent LGE-CMR and were followed for a median 3.5 years. The primary end point was SCD or aborted SCD.
  
  
  RESULTS
  Overall, 471 patients (66.2%) had myocardial fibrosis (median 5.9% of left ventricular mass, IQR: 2.2-13.3). Twenty-two (3.1%) reached the primary end point. The extent but not the presence of fibrosis was a significant univariable predictor of the primary end point (HR per 5% LGE: 1.24, 95% CI 1.06 to 1.45; p=0.007 and HR for LGE: 2.69, 95% CI 0.91 to 7.97; p=0.073, respectively). However, on multivariable analysis, only LV-EF remained statistically significant (HR: 0.92, 95% CI 0.89 to 0.95; p<0.001). For the secondary outcome of cardiovascular mortality/aborted SCD, the presence and the amount of fibrosis were significant predictors on univariable but not multivariable analysis after adjusting for LV-EF and non-sustained ventricular tachycardia.
  
  
  CONCLUSIONS
  The amount of myocardial fibrosis was a strong univariable predictor of SCD risk. However, this effect was not maintained after adjusting for LV-EF. Further work is required to elucidate the interrelationship between fibrosis and traditional predictors of outcome in HCM.
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Tevfik F
authors: TF
authors: Ismail
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Andrew
authors: A
authors: Jabbour
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors: Ankur
authors: A
authors: Gulati
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Amy
authors: A
authors: Mallorie
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Sadaf
authors: S
authors: Raza
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Thomas E
authors: TE
authors: Cowling
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Bibek
authors: B
authors: Das
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Jahanzaib
authors: J
authors: Khwaja
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors: Francisco D
authors: FD
authors: Alpendurada
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors: Ricardo
authors: R
authors: Wage
authors: R-Squared Statistics, London, UK.
authors: Michael
authors: M
authors: Roughton
authors: Institute of Cardiovascular Science, University College London, London, UK.
authors: William J
authors: WJ
authors: McKenna
authors: Institute of Cardiovascular Science, University College London, London, UK.
authors: James C
authors: JC
authors: Moon
authors: West Hertfordshire Hospitals NHS Trust, Hertfordshire, UK.
authors: Amanda
authors: A
authors: Varnava
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Department of Cardiology, South London Healthcare NHS Trust, London, UK.
authors: Carl
authors: C
authors: Shakespeare
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Martin R
authors: MR
authors: Cowie
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Stuart A
authors: SA
authors: Cook
authors: Institute of Cardiovascular Science, University College London, London, UK.
authors: Perry
authors: P
authors: Elliott
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK.
authors: Rory
authors: R
authors: O'Hanlon
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Dudley J
authors: DJ
authors: Pennell
authors: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, UK Imperial College London, London, UK.
authors: Sanjay K
authors: SK
authors: Prasad
mesh_terms: D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D016009:Chi-Square Distribution; D003287:Contrast Media; D016757:Death, Sudden, Cardiac / Q000209:etiology* / Q000473:pathology / Q000517:prevention & control; D018572:Disease-Free Survival; D005260:Female; D005355:Fibrosis; D019786:Gadolinium DTPA; D006801:Humans; D053208:Kaplan-Meier Estimate; D008131:London; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009206:Myocardium / Q000473:pathology*; D009942:Organometallic Compounds; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D011446:Prospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D062606:Tertiary Care Centers; D013997:Time Factors
chemical_list: D003287:Contrast Media; D009942:Organometallic Compounds; C090600:gadobutrol; D019786:Gadolinium DTPA