AL and TTR cardiac amyloid has a median survival of only 6-8 months, untreated. Orthotopic heart transplant (OHT) followed by stem cell transplant (SCT) for AL-amyloid or orthotopic liver transplant (OLT) for TTR-amyloid have proven successful in small studies. Given the rare nature of these diseases, a large, multi-center effort to describe these patients and their outcomes has been established.
Seven academic medical centers from the US and Europe are have compiled demographic, hemodynamic and organ-involvement data as well as OHT treatment strategies for AL and TTR amyloid. Information is currently available for 79 patients, 65 AL and 14 TTR amyloid patients.
Of the 65 AL-amyloid patients, 77% were eligible for OHT and 68% survived to OHT. 82% survived to SCT and 57% are still alive. Among the TTR amyloid patients, 100% underwent OHT. 62% underwent OHT only, 29% underwent OHT followed by OLT and 14% underwent concurrent OHT/OLT. 100% of the OHT-only patients are alive. 50% of both concurrent OHT/OLT and OHT followed by OLT patients are alive.
In AL amyloid, there was no difference in demographics, hemodynamics or extra-cardiac involvement between those that survived to OHT and those that died awaiting OHT. Survivors to OHT had lower % plasma cells (p=0.03), more lambda predominant disease (p=0.01) and a higher light chain ratio (p=0.02). Survivors to OHT had a longer time from onset of cardiac symptoms to OHT/death (p=0.01) and longer time from amyloid diagnosis (p=0.01) to OHT/death. Time on the organ donor waiting list did not differ between those that survived to OHT and those that did not.
This multi-center effort represents the largest collection of AL and TTR cardiac amyloidosis to date. We present data on 79 patients from 3 centers. For AL amyloid patients, OHT+SCT and for TTR amyloid, either OHT alone or OHT+OLT offer improved survival. In AL amyloid, hematologic parameters and a slow rate of amyloid progression appear to be the biggest determinants of survival.
To cite this abstract in AMA style:Gilstrap L, Niehaus E, Maurer M, Feltrin G, Estep J, Witteles R, Zucker M, Baran D, Kushwaha S, Seldin D, Semigran M. Multi-Center, International Registry of Cardiac Transplantation for Light Chain (AL) and Transthyreitin (TTR) Amyloidosis, A [abstract]. Am J Transplant. 2013; 13 (suppl 5). https://atcmeetingabstracts.com/abstract/multi-center-international-registry-of-cardiac-transplantation-for-light-chain-al-and-transthyreitin-ttr-amyloidosis-a/. Accessed April 10, 2020.
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