In addition, these patients are not significantly represented in the INTERMACS registry #medical randurls[1|1|,|CHEM1|]# to provide definitive recommendations on the safety and efficacy of LVAD, biventricular assist device (BiVAD), or total artificial heart therapy as a bridge to transplantation or destination therapy.19 An established risk factor for post-LVAD morbidity and mortality relates to pre-existing right-sided heart failure reflected by a high right-atrial

Inhibitors,research,lifescience,medical pressure.20, 21 Right-sided heart failure is a not uncommon cardiac manifestation in patients with end-stage cardiac amyloidosis that potentially equates to higher early postoperative risk compared to patients with nonamyloid dilated advanced cardiomyopathies. Ideal mechanical circulatory support options for patients with end-stage cardiac amyloidosis who have biventricular failure include BiVADs and the total artificial heart.22 Overall, there is a paucity of data regarding the benefits versus risks of biventricular circulatory support as a bridge Inhibitors,research,lifescience,medical to heart transplantation in patients

with end-stage Inhibitors,research,lifescience,medical systemic amyloidosis. Moreover, the use of biventricular mechanical support as destination therapy not linked to heart transplantation is associated overall with a poor 1-year survival (less than 50%).20 Heart Transplantation The major risk associated with heart transplantation for patients with end-stage cardiac amyloidosis is progression in other major organ systems, including recurrence in the cardiac allograft leading to decreased l- and 5-year post-transplant survival.23,24 Early transplant experience from the United Kingdom in 24 cases (the majority due to AL amyloidosis) without adjunctive Inhibitors,research,lifescience,medical chemotherapy showed a dismal 1- and 5-year survival of 50% and 20%, respectively.23 Inhibitors,research,lifescience,medical Compared to the current

U.S. national post-heart-transplant benchmarks provided by the SRTR (1-year survival around 89%, 5-year survival around 75%), heart transplantation for cardiac amyloidosis historically has been associated with the poorer post-transplant survival.25 However, the implementation of light-chain reductive chemotherapy and post-heart-transplant autologous hematopoietic stem cell transplant (ASCT) has improved post-heart-transplant no outcome for patients with cardiac amyloidosis. Based on the United Kingdom experience, post-heart-transplant reductive chemotherapy has improved survival to 71% at 1 year.23 ASCT, the ultimate intervention aimed to create remission of the underlying bone marrow plasma dyscrasia, has been used by a few centers,26-28 including ours, to potentially improve long-term post-heart-transplant survival. The Mayo Clinic group reported their experience with ASCT 6 months post-OHT in 11 patients with AL amyloidosis, with a survival rate of 82% and 65% at 1 and 5 years, respectively.