title: Survival After Heart Transplantation in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy.
abstract: BACKGROUND
  Outcomes of arrhythmogenic right ventricular cardiomyopathy (ARVC) patients after heart transplantation have not been well studied. Diagnostic criteria were established in 1994 and subsequently revised in 2010. We sought to better characterize this population in a national cohort.
  
  
  METHODS
  A total of 35,138 heart transplant-only recipients were identified from the United Network for Organ Sharing (UNOS) Thoracic Registry (1994-2011); 73 had ARVC. The non-ARVC group included ischemic cardiomyopathy, restrictive cardiomyopathy, dilated cardiomyopathy, hypertrophic cardiomyopathy, and other. Survival was censored at 12 years. Multivariate Cox proportional hazard regression analysis was adjusted for age, sex, DM, race, ischemia time, dialysis, life support, wait time, and HLA mismatch.
  
  
  RESULTS
  There were 73 ARVC and 35,065 non-ARVC patients. The ARVC cohort was associated with less ventricular assist device use (P = .001) and significantly decreased pulmonary arterial and capillary wedge pressures (P < .001). Survivals at 1, 5, and 10 years were, respectively, ARVC 87%, 81%, and 77%, and non-ARVC 87%, 72%, and 53% (log rank P = .07). The ARVC unadjusted hazard ratio for all-cause mortality was 0.59 (95% confidence interval [CI] 0.34-1.04; P = .073). Multivariate analysis yielded a hazard ratio of 0.68 (95% CI 0.35-1.30; P = .25). ARVC survival was similar to restrictive, hypertrophic, and dilated cardiomyopathies and significantly better than ischemic cardiomyopathy.
  
  
  CONCLUSIONS
  This is the largest reported series of ARVC after heart transplantation, of which 11% were pediatric. Survival was similar to the non-ARVC cohort, with improved survival over ischemic and restrictive etiologies.
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California. Electronic address: edepasquale@mednet.ucla.edu.
authors.forename: Eugene C
authors.initials: EC
authors.lastname: DePasquale
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Richard K
authors.initials: RK
authors.lastname: Cheng
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Mario C
authors.initials: MC
authors.lastname: Deng
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Ali
authors.initials: A
authors.lastname: Nsair
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: William J
authors.initials: WJ
authors.lastname: McKenna
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Gregg C
authors.initials: GC
authors.lastname: Fonarow
authors.affiliation: UCLA Advanced Heart Failure, Heart Transplantation & Mechanical Circulatory Support Program, David Geffen School of Medicine at UCLA, Los Angeles, California.
authors.forename: Daniel L
authors.initials: DL
authors.lastname: Jacoby
mesh_terms: D000328:Adult; D000367:Age Factors; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000401:mortality* / Q000601:surgery*; D016022:Case-Control Studies; D002423:Cause of Death; D016001:Confidence Intervals; D005260:Female; D016027:Heart Transplantation / Q000379:methods* / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D016016:Proportional Hazards Models; D012016:Reference Values; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D016019:Survival Analysis; D013997:Time Factors; D014481:United States
keywords: Arrhythmogenic right ventricular cardiomyopathy; cardiomyopathy; heart transplantation; outcomes