Cause of death analysis and temporal trends in survival after liver transplantation for transthyretin familial amyloid polyneuropathy.
Vincent AlgalarrondoTeresa AntoniniMarie ThéaudinDenis ChemlaAnouar BenmalekDenis CastaingCécile CauquilFrançois RouzetDelphine MikaEric DuongSylvie DinanianLudivine EliahouDominique Le GuludecDidier SamuelDavid AdamsMichel S SlamaPublished in: Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis (2019)
Over a median follow up of 5.9 years, 84 patients died. The rate of death was higher in the first year following LT than thereafter (13.0 vs. 4.3 ± 1.8%/year; p = .004). Cardiac events ranked as the leading cause of death (C: 38%), followed by infections (I: 24%), graft complications (G: 17%), end stage amyloidosis, stroke and others (ASO: 7% each). Deaths due to graft complications and infections (GI) occurred earlier than those due to end stage amyloidosis and stroke. Death prediction was less accurate for GI-related mortality than for other causes, which blunted the accuracy of the early-term risk prediction scores. Conclusions In ATTR amyloidosis, cardiac events were the leading cause of death after liver transplantation. Close preoperative evaluation allowed for accurate mid-term prediction of mortality, but the high rate of graft complications and infections blunted the early-term risk prediction.