Abstract

<h3>Purpose</h3> Cardiac amyloidosis (CA) results from myocardial transthyretin (ATTR) or immunoglobulin light chain (AL) deposition and is associated with high mortality. Heart transplant (HT) has emerged as an option for patients (pts) with advanced disease. We present long-term survival data following HT for pts with CA. <h3>Methods</h3> All adult HT pts at our center between 1/1/2012 and 12/31/2020 were included. Heart explant pathology reports were reviewed for evidence of amyloid deposition based on Congo red staining. ATTR subtyping was confirmed with mass spectrometry analysis. Kaplan-Meier survival analysis was computed using a log-rank test for between-group differences. <h3>Results</h3> 63 pts with CA [AL (17), wild type - ATTR (20), variant - ATTR (24), amyloidosis leukocyte chemotactic factor 2 (1), beta 2 microglobulin (1)] underwent HT during the study period. Pts with CA were older (65.4 vs. 54.2), more likely to be male (90% vs. 70%), and less likely to be white (56% vs. 71%) compared to pts without CA. 9 ATTR pts (21%) and 5 AL patients (29%) underwent dual organ transplant. 5 patients with AL amyloidosis underwent subsequent autologous stem cell transplant (ASCT). Post-HT survival at 5 years was similar for pts with AL amyloidosis, ATTR amyloidosis, and other HT patients (n = 911) (p=0.67). AL and variant-ATTR pts had numerically lower survival than wild type - ATTR patients. Cause of death for pts with AL CA included primary graft dysfunction (1), complications related to ASCT (1), and anoxic brain injury (1). For pts with ATTR CA, cause of death included liver graft rejection (1), multi organ failure (1), respiratory failure (1), decompensated heart failure (1), and acute myocardial infarction (1). <h3>Conclusion</h3> Pts with CA that meet criteria for HT experience survival comparable to that for other indications. HT remains a viable option for CA pts with advanced disease, despite older age at the time of HT.

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