Outcomes after Cardiac Transplant for Wild Type Transthyretin Amyloidosis

Background The true prevalence of heart failure (HF) 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 HF 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 mmHg) prior to 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. REPRINTS AND CORRESPONDENCE: Andrew N. Rosenbaum, M.D., Mayo Clinic Rochester, 200 1st St. SW, Rochester, MN 55905. Phone#: +15072842511. Fax#: +1 5072840161. Email: Rosenbaum.Andrew@mayo.edu PI: Brooks S. Edwards: Phone#: +15072663841 Email: Edwards.Brooks@mayo.edu AUTHORSHIP: Andrew N Rosenbaum: Participated in research design, writing of the paper, performance of the research, and data analysis Omar F. Abou Ezzeddine: Participated in writing of the paper and data analysis Martha Grogan: Participated in research design, writing of the paper, and data analysis Angela Dispenzieri: Participated in writing of the paper and data analysis Sudhir Kushwaha: Participated in writing of the paper and data analysis Alfredo Clavell: Participated in writing of the paper and data analysis Richard C. Daly: Participated in writing of the paper and data analysis Brooks S Edwards: Participated in research design, writing of the paper, and data analysis DISCLOSURES: The authors declare no conflicts of interest SOURCES OF FUNDING: No funding was provided for the current study Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

from #ORL-AlexandrosSfakianakis via ola Kala on Inoreader https://ift.tt/2HkLCN6