Amyloidosis and Heart Transplantation in a New Era
- PMID: 39775986
- DOI: 10.1111/ctr.70070
Amyloidosis and Heart Transplantation in a New Era
Abstract
Background: The prognosis in patients with advanced cardiac amyloidosis (CA) remains poor.
Objectives: We sought to describe survival post heart transplantation (HT) in amyloid compared with non-amyloid recipients, highlight waitlist times within the new allocation system across three Organ Procurement and Transplantation Network (OPTN) regions, and describe multiorgan transplantation (MOT) in hereditary amyloidosis.
Methods: This is a retrospective review of end-stage CA patients who underwent HT at Mayo Clinic from January 2007 to December 2020. Wait time was compared in the new versus old OPTN allocation era starting December 18, 2018 by Wilcoxon rank sum test. All-cause mortality for those with and without CA was compared using Kaplan-Meier estimates with log rank analysis, censoring December 16, 2022.
Results: Fifty-five patients with CA underwent HT between 2007 and 2020, 8 light chain amyloidosis (AL) (14.5%), 28 hereditary transthyretin (ATTRv) (50.9%), 17 wildtype transthyretin (ATTRwt) (30.9%), and 2 hereditary apolipoprotein A1 (AApoA1) amyloidosis patients (3.6%). No significant difference in overall survival post-transplant was seen in amyloid compared with non-amyloid (p = 0.816). Median time to HT was shorter in the new system, 45 days (IQR 24, 78) versus 174 days (IQR 76.5, 483.5), p = 0.006. There was a decline in MOT in hereditary amyloidosis over time with the concomitant rise in disease-targeted therapies.
Conclusions: HT survival in CA patients was similar to non-amyloid patients. The new allocation system benefits this cohort with shorter wait times. There is less MOT in hereditary amyloidosis with increased utilization of disease-targeted therapy.
Keywords: cardiac amyloidosis; heart transplantation; multiorgan transplantation; survival.
© 2025 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
References
-
- M. A. Gertz, “Immunoglobulin Light Chain Amyloidosis: 2018 Update on Diagnosis, Prognosis, and Treatment,” American Journal of Hematology 93 (2018): 1169–1180.
-
- J. N. Buxbaum, A. Dispenzieri, D. S. Eisenberg, et al., “Amyloid Nomenclature 2022: Update, Novel Proteins, and Recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee,” Amyloid 29 (2022): 213–219.
-
- F. L. Ruberg, M. Grogan, M. Hanna, J. W. Kelly, and M. S. Maurer, “Transthyretin Amyloid Cardiomyopathy: JACC State‐of‐the‐Art Review,” Journal of the American College of Cardiology 73 (2019): 2872–2891.
-
- J. R. Mikhael, S. R. Schuster, V. H. Jimenez‐Zepeda, et al., “Cyclophosphamide‐Bortezomib‐Dexamethasone (CyBorD) Produces Rapid and Complete Hematologic Response in Patients With AL Amyloidosis,” Blood 119 (2012): 4391–4394.
-
- E. Kastritis, G. Palladini, M. C. Minnema, et al., “Daratumumab‐Based Treatment for Immunoglobulin Light‐Chain Amyloidosis,” New England Journal of Medicine 385 (2021): 46–58.
MeSH terms
LinkOut - more resources
Full Text Sources
Medical
Research Materials