title: Long term outcomes of cardiac transplant for immunoglobulin light chain amyloidosis: The Mayo Clinic experience.
abstract: OBJECTIVE
  To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis.
  
  
  METHODS
  The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness > 15 mm, ejection fraction < 40%. Selection guidelines for heart transplant included age < 60 years, absence of multiple myeloma and significant extra-cardiac organ involvement. Baseline characteristics including age, gender, organ involvement, and New York Heart Association functional class were recorded. Laboratory data, waiting time until heart transplant, and type of treatment of the underlying plasma cell disorder were recorded. Survival from the time of OHT was calculated using Kaplan-Meier survival curves. Survival of patients undergoing OHT for AL was compared to that of non-amyloid patients undergoing OHT during the same time period.
  
  
  RESULTS
  Twenty-three patients (median age 53 years) with AL received OHT. There were no deaths in the immediate perioperative period. Twenty patients have died post OHT. For the entire cohort, the median overall survival was 3.5 years (95%CI: 1.2, 8.2 years). The 1-year survival post OHT was 77%, the 2-year survival 65%, and the 5-year survival 43%. The 5-year survival for non-amyloid patients undergoing OHT during the same era was 85%. Progressive amyloidosis contributed to death in twelve patients. Of those without evidence of progressive amyloidosis, the cause of death included complications of autologous hematopoietic stem cell transplantation for 3 patients, post-transplant lymphoproliferative disorder for 2 patients; and for the remaining one death was related to each of the following causes: acute rejection; cardiac vasculopathy; metastatic melanoma; myelodysplastic syndrome; and unknown. Eight patients had rejection at a median of 1.8 mo post OHT (range 0.4 to 4.9 mo); only one patient died of rejection. Median survival of seven patients who achieved a complete hematologic response to either chemotherapy or autologous hematopoietic stem cell transplantation was 10.8 years.
  
  
  CONCLUSIONS
  Our data demonstrate that long term survival after heart transplant is feasible in AL patients with limited extra-cardiac involvement who achieve complete hematologic response.
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Martha
authors.initials: M
authors.lastname: Grogan
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Morie
authors.initials: M
authors.lastname: Gertz
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Arleigh
authors.initials: A
authors.lastname: McCurdy
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Lindsey
authors.initials: L
authors.lastname: Roeker
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Robert
authors.initials: R
authors.lastname: Kyle
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Sudhir
authors.initials: S
authors.lastname: Kushwaha
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Richard
authors.initials: R
authors.lastname: Daly
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Joseph
authors.initials: J
authors.lastname: Dearani
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Richard
authors.initials: R
authors.lastname: Rodeheffer
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Robert
authors.initials: R
authors.lastname: Frantz
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Martha
authors.initials: M
authors.lastname: Lacy
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Hayman
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Christopher
authors.initials: C
authors.lastname: McGregor
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Brooks
authors.initials: B
authors.lastname: Edwards
authors.affiliation: Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States.
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
keywords: Amyloidosis; Autologous stem cell transplantation; Chemotherapy; Heart failure; Heart transplantation