title: Predictors of survival to orthotopic heart transplant in patients with light chain amyloidosis.
abstract: BACKGROUND
  Orthotopic heart transplant (OHT), followed by myeloablative chemotherapy and autologous stem cell transplant (ASCT), has been successful in the treatment of amyloid light-chain (AL) cardiac amyloidosis. The purpose of this study was to identify predictors of survival to OHT in patients with end-stage heart failure due to AL amyloidosis and compare post-OHT survival of cardiac amyloid patients with survival of other cardiomyopathy patients undergoing OHT.
  
  
  METHODS
  From January 2000 to June 2011, 31 patients with end-stage heart failure secondary to AL amyloidosis were listed for OHT at Massachusetts General Hospital. Univariate and multivariate regression analyses identified predictors of survival to OHT. Kaplan-Meier analysis compared survival between the Massachusetts General Hospital amyloidosis patients and non-amyloid cardiomyopathy patients from the Scientific Registry of Transplant Recipients (SRTR).
  
  
  RESULTS
  Low body mass index was the only predictor of survival to OHT in patients with end-stage heart failure caused by cardiac amyloidosis. Survival of cardiac amyloid patients who died before receiving a donor heart was only 63 ± 45 days after listing. Patients who survived to OHT received a donor organ at 53 ± 48 days after listing. Survival of AL amyloidosis patients on the waiting list was less than patients on the waiting list for all other non-amyloid diagnoses. The long-term survival of amyloid patients who underwent OHT was no different than the survival of non-amyloid, restrictive (p = 0.34), non-amyloid dilated (p = 0.34), or all non-amyloid cardiomyopathy patients (p = 0.22) in the SRTR database.
  
  
  CONCLUSIONS
  Amyloid patients who survive to OHT, followed by ASCT, have a survival rate similar to other cardiomyopathy patients undergoing OHT; however, 35% of the patients died awaiting OHT. The only predictor of survival to OHT in AL amyloidosis patients was a low body mass index, which correlated with a shorter time on the waiting list. To optimize the survival of these patients, access to donor organs must be improved.
authors.affiliation: Division of Cardiology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Lauren
authors.initials: L
authors.lastname: Gray Gilstrap
authors.affiliation: Division of Cardiology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Emily
authors.initials: E
authors.lastname: Niehaus
authors.affiliation: Division of Cardiology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Rajeev
authors.initials: R
authors.lastname: Malhotra
authors.affiliation: Division of Cardiology, Department of Medicine, Johns Hopkins Medical Institutes, Baltimore, Maryland.
authors.forename: Van-Khue
authors.initials: VK
authors.lastname: Ton
authors.affiliation: Cardiology Service, Department of Medicine, Brooke Army Medical Center, Fort Sam Houston, Texas.
authors.forename: James
authors.initials: J
authors.lastname: Watts
authors.affiliation: Section of Hematology and Oncology, Amyloidosis Center, Department of Medicine, Boston University Medical Center, Boston, Massachusetts.
authors.forename: David C
authors.initials: DC
authors.lastname: Seldin
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Joren C
authors.initials: JC
authors.lastname: Madsen
authors.affiliation: Division of Cardiology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts. Electronic address: msemigran@partners.org.
authors.forename: Marc J
authors.initials: MJ
authors.lastname: Semigran
mesh_terms: D000686:Amyloidosis / Q000150:complications*; D015992:Body Mass Index; D009202:Cardiomyopathies / Q000150:complications*; D005260:Female; D006331:Heart Diseases / Q000150:complications*; D006333:Heart Failure / Q000209:etiology* / Q000401:mortality*; D016027:Heart Transplantation; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012044:Regression Analysis; D012189:Retrospective Studies; D033581:Stem Cell Transplantation; D015996:Survival Rate; D013997:Time Factors; D014850:Waiting Lists
keywords: autologous stem cell transplant; body mass index; light-chain cardiac amyloidosis; orthotopic heart transplantation; survival