Search

The optimal immunosuppressive therapy (IST) for patients with AL amyloid undergoing orthotopic heart transplantation (OHT) is not well defined. Our study aims to present the IST and outcomes after OHT in patients with cardiac amyloidosis at a large quaternary university hospital. In a retrospective chart review, we performed a case series of all patients with AL amyloid who underwent OHT from Jan 2017 to Oct 2020. We assessed their IST and outcomes of survival, infection and rejection. We identified 7 patients with AL amyloidosis who underwent OHT. Three patients (43%) had Mayo Stage 3, and 4 (57%) patients had stage 4. Autologous stem cell transplant (ASCT) was performed in 4 patients with a median time from OHT to ASCT of 429 days. In patients who had bone marrow biopsy, amyloid was present in 21% and the mean plasma cells visualized by immunohistochemistry was 4.41%. IST after OHT consisted of a calcineurin inhibitor, antimetabolite and steroids initially. Some required treatment with bortezomib/daratumumab for sub-optimal duration or hematologic response to AL amyloid therapies prior to OHT at which time antimetabolite was stopped. Median time to antimetabolite withdrawal was 317 days. Median times to introduction of daratumumab and bortezomib after OHT were 283 and 388 days, respectively. Figure 1 displays the time series of IST. Renal involvement was the more prevalent extracardiac organ (57%), followed by gastrointestinal (43%), carpal tunnel (29%), and peripheral neuropathy (29%). We did not observe any mortality, graft failure, episodes of acute cellular or antibody-mediated rejection. One patient had clostridium difficile infection who was admitted for monitoring. In those that require ongoing AL amyloid therapies following OHT, it may be safe to discontinue antimetabolite in efforts to minimize complications from over-immunosupression without compromising risk of rejection. Personalized IST in this traditionally high-risk population should be considered. [ABSTRACT FROM AUTHOR]