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Published on: February 14, 2022
Outcomes after heart transplantation for amyloid cardiomyopathy in the modern era
1Division of Cardiovascular Medicine and Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA.
Insights
Heart transplantation (HT) offers a feasible option for amyloid cardiomyopathy (ACM). Careful patient selection and treatment of amyloid light chains in AL amyloidosis patients lead to acceptable short-to-intermediate term outcomes.
Area of Science:
- Cardiology
- Hematology
- Transplantation Medicine
Background:
- Amyloid cardiomyopathy (ACM) is a progressive disease affecting the heart.
- Heart transplantation (HT) is a potential treatment for end-stage ACM.
- Outcomes for ACM patients undergoing HT require further investigation.
Purpose of the Study:
- To evaluate the outcomes of heart transplantation (HT) in patients with amyloid cardiomyopathy (ACM).
- To assess the feasibility and safety of HT for end-stage ACM.
- To analyze the impact of amyloid subtype and treatment on HT outcomes.
Main Methods:
- Retrospective review of 19 ACM patients who underwent HT between 2008 and 2013.
- Analysis of amyloid subtypes (AL and ATTR), pre- and post-HT treatments, and patient outcomes.
- Assessment of graft amyloidosis recurrence and overall survival.
Main Results:
- 19 ACM patients underwent HT, representing 9.4% of all HTs during the period.
- AL amyloidosis patients showed significant reduction in free light chain levels with chemotherapy.
- Survival rate was 89.5% after a median follow-up of 380 days, with one case of graft recurrence.
Conclusions:
- Heart transplantation is a feasible option for carefully selected end-stage ACM patients.
- Aggressive management of amyloidogenic light chains is crucial for AL amyloidosis patients undergoing HT.
- Acceptable short-to-intermediate term outcomes can be achieved in ACM patients receiving HT.
Abstract:
We conducted a review of patients undergoing heart transplantation (HT) at our institution for amyloid cardiomyopathy (ACM) between 2008 and 2013. Complete follow-up was available for all patients. Nineteen patients with ACM underwent HT during the study period, accounting for 9.4% of all HT performed at our institution during this period. Amyloid subtype was light chain (AL) in 9 patients and transthyretin (ATTR) in 10 (2 wild-type, 7 familial, 1 unknown). Eight of nine patients with AL amyloidosis began chemotherapy prior to HT, six have resumed chemotherapy since HT, and five have undergone autologous stem cell transplantation. Most recent free light chain levels in AL patients decreased by a median of 85% from peak values. Only one patient developed recurrent graft amyloidosis, occurring at 3.5 years post-HT and asymptomatic. After a median follow-up of 380 days, 17 (89.5%) patients are alive. To our knowledge, this is the largest single-center series reported of ACM patients undergoing HT in the modern era. Our results suggest that acceptable outcomes following HT can be achieved in the short-to-intermediate term and that this is a feasible option for end-stage ACM with careful patient selection and aggressive control of amyloidogenic light chains in AL patients.
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