title: Prevention of sudden death in hypertrophic cardiomyopathy: bridging the gaps in knowledge.
abstract: Sudden cardiac death (SCD) is the most devastating complication of hypertrophic cardiomyopathy (HCM). Although the annual rate of SCD in the general HCM population is <1% per year according to contemporary series, there is still a small subset of patients who are at increased risk of SCD. The greatest challenge in the management of HCM is identifying those at increased risk as an implantable cardioverter defibrillator is a potentially life-saving therapy. In this review, we sought to summarize the available data on SCD in HCM and provide a clinical perspective on the current differing and somewhat conflicting European and American recommendations on risk stratification, with balanced guidance with regards to rational clinical decision making. Additionally, we sought to learn more on the actual implementation of the guidelines by HCM experts worldwide.
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, ON, Canada.
authors.forename: Adaya
authors.initials: A
authors.lastname: Weissler-Snir
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, ON, Canada.
authors.forename: Arnon
authors.initials: A
authors.lastname: Adler
authors.affiliation: Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, UK.
authors.forename: Lynne
authors.initials: L
authors.lastname: Williams
authors.affiliation: Division of Cardiology, Cardiovascular Centre, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Christiane
authors.initials: C
authors.lastname: Gruner
authors.affiliation: Division of Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University Health Network, Toronto, ON, Canada.
authors.forename: Harry
authors.initials: H
authors.lastname: Rakowski
mesh_terms: D000328:Adult; D000367:Age Factors; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000453:epidemiology / Q000235:genetics; D000066491:Clinical Decision-Making; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D015444:Exercise / Q000502:physiology; D005838:Genotype; D006322:Heart Aneurysm / Q000209:etiology; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D018810:Magnetic Resonance Angiography; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D018570:Risk Assessment / Q000379:methods; D012307:Risk Factors; D013575:Syncope / Q000209:etiology; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000628:therapy; D018487:Ventricular Dysfunction, Left / Q000209:etiology; D014693:Ventricular Fibrillation / Q000209:etiology / Q000628:therapy; D014694:Ventricular Outflow Obstruction / Q000209:etiology
keywords: Hypertrophic cardiomyopathy; Implantable cardioverter defibrillator; Risk Stratification; Sudden cardiac death