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Outcomes After Cardiac Transplant for Wild Type Transthyretin Amyloidosis

Rosenbaum, Andrew N., MD1; AbouEzzeddine, Omar F., MD, CM, MS1; Grogan, Martha, MD1; Dispenzieri, Angela, MD2,3; Kushwaha, Sudhir, MD1,3; Clavell, Alfredo, MD1,3; Daly, Richard C., MD4; Edwards, Brooks S., MD1,4

doi: 10.1097/TP.0000000000002240
Original Clinical Science—General

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−1·min−1) 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.

This is the first report of a series of wild type transthyretin amyloidosis (ATTRwt) patients receiving cardiac transplant in which excellent outcomes are demonstrated and systemic manifestations of ATTRwt may develop posttransplantation although cardiac death is averted.

1 Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.

2 Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN.

3 William J von Liebig Center for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, MN.

4 Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN.

Received 11 January 2018. Revision received 13 March 2018.

Accepted 5 April 2018.

The authors declare no funding or conflicts of interest.

A.N.R. participated in research design, writing of the article, performance of the research, and data analysis. O.F.A.E. participated in writing of the article and data analysis. M.G. participated in research design, writing of the article, and data analysis. A.D. participated in writing of the article and data analysis. S.K. participated in writing of the article and data analysis. A.C. participated in writing of the article and data analysis. R.C.D. participated in writing of the article and data analysis. B.S.E. participated in research design, writing of the article, and data analysis.

Correspondence: Andrew N. Rosenbaum, MD, Mayo Clinic, 200 1st St, SW Rochester, MN 55905. (Rosenbaum.Andrew@mayo.edu).

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