Retrospective Study
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Transplant. Jun 24, 2016; 6(2): 380-388
Published online Jun 24, 2016. doi: 10.5500/wjt.v6.i2.380
Long term outcomes of cardiac transplant for immunoglobulin light chain amyloidosis: The Mayo Clinic experience
Martha Grogan, Morie Gertz, Arleigh McCurdy, Lindsey Roeker, Robert Kyle, Sudhir Kushwaha, Richard Daly, Joseph Dearani, Richard Rodeheffer, Robert Frantz, Martha Lacy, Suzanne Hayman, Christopher McGregor, Brooks Edwards, Angela Dispenzieri
Martha Grogan, Sudhir Kushwaha, Richard Rodeheffer, Robert Frantz, Brooks Edwards, Department of Cardiovascular Diseases, Mayo Clinic, Rochester, MN 55905, United States
Morie Gertz, Robert Kyle, Martha Lacy, Suzanne Hayman, Angela Dispenzieri, Division of Hematology, Mayo Clinic, Rochester, MN 55905, United States
Arleigh McCurdy, Department of Hematology, the Ottawa Hospital, University of Ottawa, Ottawa, ON K1H8L6, Canada
Lindsey Roeker, Department of Medicine, Brigham and Women’s Hospital, Boston, MA 02115, United States
Richard Daly, Joseph Dearani, Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, United States
Christopher McGregor, Department of Cardiothoracic Surgery, University College, London WC1E6BT, United Kingdom
Author contributions: Grogan M, Gertz M, McCurdy A, Roeker L, Edwards B and Dispenzieri A designed the research study and drafted and revised the manuscript; Kyle R, Kushwaha S, Daly R, Dearani J, Rodeheffer R, Frantz R, Lacy M, Hayman S and McGregor C contributed to data collection and critically revised the manuscript.
Institutional review board statement: The Mayo Foundation Institutional Review Board approved this study.
Informed consent statement: Patients were not required to give informed consent because of observational, retrospective nature of the study.
Conflict-of-interest statement: The authors of this manuscript have no conflicts of interest to disclose pertinent to this research.
Data sharing statement: No additional data available.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Martha Grogan, MD, Department of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, United States. grogan.martha@mayo.edu
Telephone: +1-507-2843681 Fax: +1-507-2660103
Received: January 25, 2016
Peer-review started: January 27, 2016
First decision: February 29, 2016
Revised: March 20, 2016
Accepted: April 7, 2016
Article in press: April 11, 2016
Published online: June 24, 2016
Processing time: 149 Days and 12.2 Hours
Abstract

AIM: 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.

CONCLUSION: 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.

Keywords: Heart transplantation; Autologous stem cell transplantation; Amyloidosis; Chemotherapy; Heart failure

Core tip: Heart failure due to immunoglobulin light chain (AL) amyloidosis is a devastating disease with poor prognosis. Orthotopic heart transplantation (OHT) is controversial. Twenty-three patients with AL amyloid underwent OHT at our institution over a twenty-year period. Median survival was 3.5 years following OHT. Median survival of seven patients who achieved a complete hematologic response to treatment for AL was almost 11 years. This study demonstrates that long term survival after heart transplant is feasible in AL patients with limited extra-cardiac involvement who achieve complete hematologic response.