Several small case series with sequential OHT and ASCT have revived enthusiasm about heart transplantation for patients with end-stage amyloidosis. Cardiac transplantation

in patients with AL amyloidosis without sequential ASCT is associated with a poor 3- to 5-year survival. In contrast, based on our ongoing experience as well as that reported by others,18, 27, 28 sequential OHT-ASCT improves survival measured after 1 year. Our planned waiting time after 2004 of at least 1-year post-OHT prior to ASCT is different from other reported small case series (waiting times between 6 and 9 months between OHT Inhibitors,research,lifescience,medical and ASCT) and importantly has not translated into amyloid disease recurrence in the cardiac allograft or clinically significant cardiac allograft dysfunction. However, our patient with partial remission post-ASCT did require a kidney transplant due to amyloid-related kidney progression. It is unclear if ASCT

sooner Inhibitors,research,lifescience,medical after OHT would have halted amyloid-related disease progression. Conclusion Unfortunately, patients with end-stage amyloidosis listed for heart transplantation selleck chem inhibitor continue to have an extraordinarily poor prognosis, Inhibitors,research,lifescience,medical with 50% death on the waiting list reported by others18 and similarly high at our institution (death during the evaluation process plus wait-list mortality ~ 35%). Death on the waiting list is often due to progressive biventricular failure and/or complications of Inhibitors,research,lifescience,medical systemic amyloidosis coupled with long waiting times for a donor heart. Earlier use of biventricular unlike mechanical circulatory support may be beneficial in this high-risk patient population. More importantly, perhaps earlier referral to an established amyloid center like ours Inhibitors,research,lifescience,medical may allow for earlier listing and initiation of less-invasive mechanical support (i.e., IABP support) to successfully bridge

patients to OHT followed by ASCT. At our program, we place the IABP percutaneously in the left axillary artery position to permit upright sitting and ambulation Cilengitide while waiting for OHT.30 At our center, a multidisciplinary approach including hematology and cardiovascular specialists is dedicated to promptly obtaining an exact diagnosis, initiating appropriate screening to determine the extent of end-organ involvement and, most importantly, carefully selecting patients for OHT or heart-multi-organ transplantation. In addition, after heart transplantation we use standard and newer treatments (i.e., bortezomib) in conjunction with anti-rejection therapy, all guided by our Amyloid Working Group, to minimize AL amyloid-related disease progression and to best prepare our patients to undergo ASCT for the most optimal chance at remission and improvement in long-term survival.