title: Changing outcomes after heart transplantation in patients with amyloid cardiomyopathy.
abstract: BACKGROUND
  Amyloid cardiomyopathy (ACM) is associated with a poor prognosis. Previous reports have suggested unfavorable post-heart transplant (HT) survival in this population compared with other HT recipients.
  
  
  METHODS
  Data from the United Network for Organ Sharing (UNOS) registry were used to study outcomes among ACM patients undergoing HT in the modern era (Era 2, 2008 to 2013) as compared with the historical era (Era 1, 1987 to 2007).
  
  
  RESULTS
  One hundred eighty-eight ACM patients underwent primary single-organ HT. Ninety-seven patients (51.6%) were transplanted in Era 1 and 91 (48.4%) in Era 2. ACM patients undergoing HT in Era 2 were older (p < 0.0001), had higher body mass index (p = 0.008) and longer ischemic times (p = 0.02), and were more likely to be African-American (p < 0.0001), UNOS Status 1A (p < 0.0001), male (p = 0.01) and highly sensitized (p < 0.0001) compared with those in Era 1. Compared with patients with other etiologies of restrictive cardiomyopathy (RCM; n = 339 in Era 1, n = 164 in Era 2), adjusted hazard ratios (HRs) for post-HT mortality of ACM were 2.08 (p < 0.0001) in Era 1 and 1.22 (p = not statistically significant) in Era 2. Adjusted HRs for mortality of ACM vs all other diagnoses (n = 36,334 in Era 1, n = 9,225 in Era 2) were 1.84 (p < 0.0001) in Era 1 and 1.38 (p = NS) in Era 2. Although post-HT survival did not change with time among non-ACM RCM patients, post-HT mortality was lower in Era 2 compared with Era 1 among ACM patients (HR 0.49, p = 0.03).
  
  
  CONCLUSIONS
  Although historically associated with inferior survival, post-HT outcomes in ACM patients in the modern era are now approaching those of non-ACM patients. Changes in patients' demographics suggest that this may be related to improved patient selection, including an increased proportion of patients with transthyretin ACM. HT should be considered for appropriate candidates with ACM.
authors.affiliation: Division of Cardiovascular Medicine and Stanford Amyloid Center, Stanford University School of Medicine, Stanford, California.
authors.forename: Margot K
authors.initials: MK
authors.lastname: Davis
authors.affiliation: Division of Cardiac Surgery, The Ohio State University Wexner Medical Center, Columbus, Ohio.
authors.forename: Peter H U
authors.initials: PH
authors.lastname: Lee
authors.affiliation: Division of Cardiovascular Medicine and Stanford Amyloid Center, Stanford University School of Medicine, Stanford, California. Electronic address: witteles@stanford.edu.
authors.forename: Ronald M
authors.initials: RM
authors.lastname: Witteles
mesh_terms: D000686:Amyloidosis / Q000401:mortality / Q000601:surgery*; D009202:Cardiomyopathies / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D016027:Heart Transplantation / Q000401:mortality*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology; D014850:Waiting Lists / Q000401:mortality*
keywords: UNOS; amyloidosis; cardiomyopathy restrictive; heart transplantation; immunoglobulin light chains; patient selection; transthyretin