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Updated: Feb 11, 2026
Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Outcomes After Cardiac Transplant for Wild Type Transthyretin Amyloidosis
Andrew N Rosenbaum1, Omar F AbouEzzeddine1, Martha Grogan1
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.
Insights
Heart transplant (HTx) for wild type transthyretin amyloidosis (ATTRwt) shows excellent survival and improved function. However, extracardiac ATTRwt manifestations can emerge post-transplant.
Area of Science:
- Cardiology
- Transplant Surgery
- Amyloidosis Research
Background:
- Wild type transthyretin amyloidosis (ATTRwt) is an underdiagnosed cause of heart failure.
- Limited data exists on managing advanced ATTRwt heart failure and its extracardiac progression.
Purpose of the Study:
- To evaluate outcomes of heart transplant (HTx) in patients with ATTRwt-related heart failure.
- To describe the natural history of extracardiac ATTRwt after HTx.
Main Methods:
- Retrospective cohort study of seven ATTRwt patients undergoing HTx.
- Collected comprehensive pre- and post-transplant clinical, hemodynamic, and echocardiographic data.
Main Results:
- 100% three-year survival; one late death from unrelated cancer.
- Significant improvements in oxygen consumption and 6-minute walk distance.
- Late-onset gastrointestinal and peripheral nerve involvement observed in some patients.
Conclusions:
- Cardiac transplant offers excellent outcomes for ATTRwt heart failure, averting cardiac death.
- Systemic ATTRwt manifestations may develop or progress after HTx, requiring ongoing monitoring.
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.
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