title: Hypertrophic Cardiomyopathy in Adulthood Associated With Low Cardiovascular Mortality With Contemporary Management Strategies.
abstract: BACKGROUND
  Hypertrophic cardiomyopathy (HCM) has been prominently associated with adverse disease complications, including sudden death or heart failure death and a generally adverse prognosis, with annual mortality rates of up to 6%.
  
  
  OBJECTIVE
  This study determined whether recent advances in management strategy, including implantable cardioverter-defibrillators (ICDs), heart transplantation, or other therapeutic measures have significantly improved survival and the clinical course of adult HCM patients.
  
  
  METHODS
  We addressed long-term outcomes in 1,000 consecutive adult HCM patients presenting at 30 to 59 years of age (mean 45±8 years) over 7.2±5.2 years of follow-up.
  
  
  RESULTS
  Of 1,000 patients, 918 (92%) survived to 53±9.2 years of age (range 32 to 80 years) with 91% experiencing no or only mild symptoms at last evaluation. HCM-related death occurred in 40 patients (4% [0.53%/year]) at 50±10 years from the following events: progressive heart failure (n=17); arrhythmic sudden death (SD) (n=17); and embolic stroke (n=2). In contrast, 56 other high-risk patients (5.6%) survived life-threatening events, most commonly with ICD interventions for ventricular tachyarrhythmias (n=33) or heart transplantation for advanced heart failure (n=18 [0.79%/year]). SD occurred in patients who declined ICD recommendations, had evaluations before application of prophylactic ICDs to HCM, or were without conventional risk factors. The 5- and 10-year survival rates (confined to HCM deaths) were 98% and 94%, respectively, not different from the expected all-cause mortality in the general U.S. population (p=0.25). Multivariate independent predictors of adverse outcome were younger age at diagnosis, female sex, and increased left atrial dimension.
  
  
  CONCLUSIONS
  In a large longitudinally assessed adult HCM cohort, we have demonstrated that contemporary management strategies and treatment interventions, including ICDs for SD prevention, have significantly altered the clinical course, now resulting in a low disease-related mortality rate of 0.5%/year and an opportunity for extended longevity.
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota. Electronic address: hcm.maron@mhif.org.
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
authors.affiliation: Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston, Massachusetts.
authors.forename: Ethan J
authors.initials: EJ
authors.lastname: Rowin
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: Susan A
authors.initials: SA
authors.lastname: Casey
authors.affiliation: Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston, Massachusetts.
authors.forename: Mark S
authors.initials: MS
authors.lastname: Link
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: John R
authors.initials: JR
authors.lastname: Lesser
authors.affiliation: Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston, Massachusetts.
authors.forename: Raymond H M
authors.initials: RH
authors.lastname: Chan
authors.affiliation: Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: Ross F
authors.initials: RF
authors.lastname: Garberich
authors.affiliation: Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston, Massachusetts.
authors.forename: James E
authors.initials: JE
authors.lastname: Udelson
authors.affiliation: Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston, Massachusetts.
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002312:Cardiomyopathy, Hypertrophic / Q000401:mortality* / Q000628:therapy*; D002316:Cardiotonic Agents / Q000627:therapeutic use; D016757:Death, Sudden, Cardiac / Q000453:epidemiology; D017147:Defibrillators, Implantable; D005260:Female; D006325:Heart Atria / Q000473:pathology; D006333:Heart Failure / Q000401:mortality; D016027:Heart Transplantation; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012737:Sex Factors; D020521:Stroke / Q000209:etiology; D017180:Tachycardia, Ventricular / Q000517:prevention & control; D014481:United States / Q000453:epidemiology
chemical_list: D002316:Cardiotonic Agents
keywords: heart failure; heart transplant; implantable defibrillators; sudden death