title: The value of cardiac magnetic resonance and distribution of late gadolinium enhancement for risk stratification of sudden cardiac death in patients with hypertrophic cardiomyopathy.
abstract: The presence of late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM) is associated with worse clinical outcome and the extent of LGE predicts the increased risk of sudden cardiac death (SCD). Limited data exist regarding the distribution of LGE. We attempted to verify whether the presence of LGE outside the interventricular insertion points carries additional risk for patients with HCM.
  
  
  
  In this prospective study, 328 patients with HCM, who underwent cardiac magnetic resonance (CMR) were enrolled. Five major risk factors for SCD were assessed in all patients. The median follow-up was 37 months.
  
  
  
  LGE was detected in 226 (68.9%) patients. In 70 (21.3%) patients it was present only at the interventricular insertion points - LGE (+) group, while in 156 (47.6%) it was noted in other locations - LGE (++) group. Primary endpoint defined as SCD or appropriate implantable cardioverter-defibrillator intervention occurred in 14 (4.3%) patients, one in LGE (+) and 13 in LGE (++). In multivariable analysis including five traditional risk factors and left ventricular ejection fraction <50%, only the presence of LGE outside the insertion points was a significant predictor of SCD/aborted SCD (HR 10.01, 95% CI 1.21-83.86, p=0.033). The performance of the multivariable sudden cardiac death risk model was improved by the addition of LGE (++) to the traditional risk factors (likelihood ratio p=0.005). The Kaplan-Meier curves showed better event-free survival in the LGE (-) and LGE (+) patient groups compared to the LGE (++) group.
  
  
  
  In HCM patients, presence of LGE outside interventricular insertion points is associated with increased risk of sudden cardiac death or its equivalent as well as overall mortality. Cardiac fibrosis as a substrate for SCD in HCM may be identified on CMR and serve as an imaging biomarker of increased risk.
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland. Electronic address: mklopotowski@ikard.pl.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Klopotowski
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Kukula
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland; Magnetic Resonance Unit, Institute of Cardiology, Warsaw, Poland.
authors.forename: Lukasz A
authors.initials: LA
authors.lastname: Malek
authors.affiliation: Magnetic Resonance Unit, Institute of Cardiology, Warsaw, Poland; Department of Coronary Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland.
authors.forename: Mateusz
authors.initials: M
authors.lastname: Spiewak
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Polanska-Skrzypczyk
authors.affiliation: Public Health Department, Medical University of Bialystok, Bialystok, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Jamiolkowski
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Maciej
authors.initials: M
authors.lastname: Dabrowski
authors.affiliation: Department of Cardiac Arrhythmias, Institute of Cardiology, Warsaw, Poland.
authors.forename: Rafal
authors.initials: R
authors.lastname: Baranowski
authors.affiliation: Department of Congenital Heart Diseases, Institute of Cardiology, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Klisiewicz
authors.affiliation: Department of Cardiac Surgery and Transplantation, Institute of Cardiology, Warsaw, Poland.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Kusmierczyk
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Jasinska
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Ewelina
authors.initials: E
authors.lastname: Jarmus
authors.affiliation: Department of Coronary Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Kruk
authors.affiliation: Department of Coronary Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland.
authors.forename: Witold
authors.initials: W
authors.lastname: Ruzyllo
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Adam
authors.initials: A
authors.lastname: Witkowski
authors.affiliation: Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland.
authors.forename: Lidia
authors.initials: L
authors.lastname: Chojnowska
mesh_terms: D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D003287:Contrast Media; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D017147:Defibrillators, Implantable; D018572:Disease-Free Survival; D005260:Female; D005355:Fibrosis; D005500:Follow-Up Studies; D005682:Gadolinium; D006321:Heart / Q000000981:diagnostic imaging; D006801:Humans; D053208:Kaplan-Meier Estimate; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D011237:Predictive Value of Tests; D011446:Prospective Studies; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D016277:Ventricular Function, Left
chemical_list: D003287:Contrast Media; D005682:Gadolinium
keywords: Cardiac magnetic resonance; Hypertrophic cardiomyopathy; Imaging biomarker; Late gadolinium enhancement; Sudden cardiac death