Abstract 2410: Heart Transplantation Followed by High-Dose Chemotherapy qnd Autologous Stem Cell Transplantation in Patients with Symptomatic Cardiac Light-Chain Amyloidosis
Cardiac light-chain amyloidosis is associated with poor prognosis and median survival of 6 months without therapy of the underlying plasma cell disorder. Causative therapy regimens with highest rate of complete remission consist of high-dose melphalan and autologous stem cell transplantation, however, patients with advanced cardiac involvement are deemed ineligible due to high therapy-related mortality. Improvement of cardiac status by heart transplantation (HTX) might enable this causative therapy regimen and ultimately improve prognosis. 25 patients with symptomatic cardiac AL amyloidosis were evaluated for HTX (13 women; 12 men; median age 53 [40 – 62] years). Additional symptomatic organ involvement was excluded by extensive screening. 4 – 6 month after surgery autologous stem cell transplantation was planned in patients who were not in complete remission (CR). Median time from initial evaluation to listing for HTX was 40 (13–1528) days. During evaluation 7/25 patients died from sudden death. 18/25 patients underwent HTX at a median of 26 (7–253) days later. Within 180 (124 –253) days 8/18 (44%) patients underwent high-dose chemotherapy, 1 of them before HTX. This therapy resulted in CR (n = 3/8; 38%) and partial remission (PR; n = 3/8; 38%), appraisal was too early after chemotherapy in 2/8 (25%) patients. 4/18 patients had cardiac amyloid progression early after chemotherapy despite complete remission. 2/18 (11%) are awaiting high-dose chemotherapy. During median follow-up of 391 (54 –2223) days after HTX 4/18 (22%) patients died due to rapidly progressive amyloidosis without causative chemotherapy. Major complications after HTX were related to progression of neural (n = 3) and gastrointestinal (n = 3) involvement. In two patients with PR mild amyloid deposits were found 2 years after HTX in routine biopsies without any hemodynamic deterioration. Actuarial 1-year survival was 72%. According to the present short-term results urgent heart transplantation appears suitable in symptomatic cardiac amyloidosis offering causative therapy and might improve prognosis. Due to the wide-spread organ involvement individual patient selection is mandatory.