Long Term Hematologic and Graft Outcomes After Cardiac Transplant in Al Amyloidosis

Abstract

<h3>Purpose</h3> Advances in the systemic treatment of AL amyloidosis have allowed some patients with end-stage cardiac disease to undergo heart transplantation (HT). There is limited data on long-term hematologic and graft outcomes of patients with AL amyloidosis who underwent HT. <h3>Methods</h3> Outcomes of patients with cardiac AL amyloidosis who underwent HT at our center between 2008 and 2020 were analyzed. Descriptive data on treatments and International Myeloma Working Group hematologic response after HT were included. Incidence and interval between HT and development of coronary allograft vasculopathy (CAV, ISHLT classification), incidence of graft rejection and survival were reported. <h3>Results</h3> Eight patients with cardiac AL amyloidosis were included in the study. Three (38%) patients had concurrent multiple myeloma. Median recipient age was 53 years (range 37 - 74 years) and median follow-up was 25.2 months (range 9.6 - 150 months) from HT. Median donor age was 32 years (range 19 - 61 years). Three donors were HCV NAT+. At the time of HT, 4 patients were in complete remission (CR), 1 had stable disease (SD), and 2 had partial response (PR). Three (38%) patients underwent autologous stem cell transplant (SCT) after HT; and seven (87%) received systemic therapy after HT. Five (63%) pts developed CAV (four ISHLT CAV-1 and one ISHLT CAV-3) at a median of 13.2 months after HT. At 3 years post HT, 7 (87%) patients maintained in CR or stringent CR and only 1 (12%) patient had progressive disease (PD). Three patients died during follow-up: two from CAV and one from complications of GI amyloidosis (Figure 1). <h3>Conclusion</h3> In this single center study, most patients with cardiac AL amyloidosis experienced durable CR after HT. However, a significant proportion developed CAV and almost 40% died within three years of transplant. Further studies with larger numbers of patients are required to determine the incidence of CAV in this population. The long term outcome of heart transplantation in patients with cardiac AL amyloidosis remains to be elucidated.