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Background: The true prevalence of heart failure due to wild type transthyretin amyloidosis (ATTRwt) is likely underestimated. There is a paucity of data with regard to the management of ATTRwt-related advanced heart failure and the natural history of extracardiac ATTRwt., Methods: We conducted a retrospective cohort study of patients undergoing cardiac transplant (HTx) for ATTRwt at a single institution. Comprehensive clinical data, including baseline hemodynamic and echocardiographic characteristics, and posttransplant outcomes, were obtained., Results: Seven patients with ATTRwt underwent HTx between 2007 and 2015. All patients were male with a mean age of 66 ± 9. Patients had a reduced ejection fraction (mean, 37 ± 14%) and elevated filling pressures pre-HTx (mean pulmonary capillary wedge pressure 22 ± 7 mm Hg) before HTx. Three-year survival was 100%; 1 patient died of pancreatic cancer 45 months post-HTx (1 death per 30.8 patient-years). Oxygen consumption (Δ +6.8 ± 4.9 mL·kg·min) and 6-minute walk distances (Δ +189 ± 60 m) improved. Symptomatic gastrointestinal involvement (n = 2) and peripheral nerve involvement (n = 4) by ATTRwt developed late., Conclusions: This is the first report of a series of ATTRwt patients receiving HTx in which excellent outcomes are demonstrated. Although cardiac death is averted, systemic manifestations of ATTRwt may develop posttransplantation.

Introduction: The significance of pretransplant human leukocyte antigen antibodies (HLA-Abs), especially donor-specific HLA-Abs (DSA), as detected by single antigen bead assay (SAB), is not well characterized in cardiac transplantation (CTX). We analyzed the significance of DSA detected by SAB in predicting crossmatch (XM) results and post-transplant rejection., Materials and Methods: We performed a retrospective study of 85 CTX with negative cytotoxicity XM. We tested pretransplant sera collected within 24 hours of transplantation by flow cytometric XM (FXM) and SAB. DSA identified by SAB were utilized to perform a virtual crossmatch (VXM). Positive VXM was defined as the presence of DSA at mean fluorescence intensity (DMFI)>1500. Additionally, to analyze the significance of low-level DSA weakly positive VXM was DMFI 300 to 1500. We defined a negative VXM as MFI<300. VXM results were correlated with FXM results and with posttransplant rejection., Results: Patients in the weakly positive and negative VXM had similar posttransplant rejections. DMFI>1500 correlates well with FXM results (accuracy=90%). Patients with DMFI>1500 had a higher incidence of antibody-medicated rejection (AMR; P=.0052), AMR grade I (P<.0001), cell-mediated rejection (CMR) grade>1R/1A (P=.018), and CMR grade>2R/3A (P=.057). Similarly patients with positive FXM had a higher incidence of AMR (P=.091), AMR grade 1 (P<.0001), CMR grade>1R/1A (P=.05), and CMR grade>2R/3A (P=.56)., Conclusions: In conclusion, SAB defined DMFI>1500 can be used as a surrogate for FXM. Recipients with DMFI>1500 pretransplant and positive FXM have significantly higher rates of AMR and CMR compared to recipients with DMFI<1500 or negative FXM., (Copyright © 2011 Elsevier Inc. All rights reserved.)