title: Cardiac transplantation using extended-donor criteria organs for systemic amyloidosis complicated by heart failure.
abstract: BACKGROUND
  Systemic amyloidosis complicated by heart failure is associated with high cardiovascular morbidity and mortality. Heart transplantation for patients with systemic amyloidosis is controversial due to recurrence of disease in the transplanted organ or progression of disease in other organs.
  
  
  METHODS
  All patients with systemic amyloidosis and heart failure referred for heart transplant evaluation from 1997 to 2004 were included in this retrospective cohort analysis. An interdisciplinary protocol for cardiac transplantation using extended-donor criteria organs, followed in 6 months by either high-dose chemotherapy and stem cell transplantation for patients with primary (AL) or by orthotopic liver transplantation for familial (ATTR) amyloidosis, was developed. Survival of the transplanted amyloid cohort was compared to survival of those amyloid patients not transplanted and to patients transplanted for other indications.
  
  
  RESULTS
  A total of 25 patients with systemic amyloidosis and heart failure were included in the study; 12 patients received heart transplants. Amyloid heart transplant recipients were more likely female (58% vs. 8%, P=0.02) and had lower serum creatinine (1.3+/-0.5 vs. 2.0+/-0.7 mg/dL, P=0.01) than nontransplanted amyloid patients. Survival at 1-year after heart transplant evaluation was higher among transplanted patients (75% vs. 23%) compared to patients not transplanted (P=0.001). Short-term survival posttransplant did not differ between transplanted amyloid patients and contemporaneous standard and extended-donor criteria heart transplant patients (P=0.65).
  
  
  CONCLUSIONS
  Cardiac transplantation for amyloid patients with extended-donor criteria organs followed by either stem cell or liver transplantation is associated with improved survival compared to patients not transplanted. Short- to intermediate-term survival is similar to patients receiving heart transplantation for other indications. This clinical management strategy provides cardiac amyloid patients a novel therapeutic option.
authors.affiliation: Department of Medicine, Divisions of Cardiology, Columbia University Medical Center, New York, NY 10032, USA. msm10@columbia.edu
authors.forename: Mathew S
authors.initials: MS
authors.lastname: Maurer
authors.forename: Amresh
authors.initials: A
authors.lastname: Raina
authors.forename: Charles
authors.initials: C
authors.lastname: Hesdorffer
authors.forename: Rachel
authors.initials: R
authors.lastname: Bijou
authors.forename: Paolo
authors.initials: P
authors.lastname: Colombo
authors.forename: Mario
authors.initials: M
authors.lastname: Deng
authors.forename: Ronald
authors.initials: R
authors.lastname: Drusin
authors.forename: Jennifer
authors.initials: J
authors.lastname: Haythe
authors.forename: Evelyn
authors.initials: E
authors.lastname: Horn
authors.forename: Sun Hi
authors.initials: SH
authors.lastname: Lee
authors.forename: Charles
authors.initials: C
authors.lastname: Marboe
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Naka
authors.forename: Larry
authors.initials: L
authors.lastname: Schulman
authors.forename: Brian
authors.initials: B
authors.lastname: Scully
authors.forename: Peter
authors.initials: P
authors.lastname: Shapiro
authors.forename: Kenneth
authors.initials: K
authors.lastname: Prager
authors.forename: Jai
authors.initials: J
authors.lastname: Radhakrishnan
authors.forename: Susan
authors.initials: S
authors.lastname: Restaino
authors.forename: Donna
authors.initials: D
authors.lastname: Mancini
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000601:surgery*; D028226:Amyloidosis, Familial / Q000150:complications / Q000601:surgery; D003404:Creatinine / Q000097:blood; D005260:Female; D006333:Heart Failure / Q000209:etiology / Q000601:surgery*; D016027:Heart Transplantation / Q000379:methods* / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D033581:Stem Cell Transplantation; D016019:Survival Analysis; D014019:Tissue Donors; D016896:Treatment Outcome
chemical_list: D003404:Creatinine