Selection of hereditary transthyretin amyloid patients for liver transplantation: the Swedish experience

Abstract

Liver transplantation (LTx) is currently an accepted treatment for hereditary transthyretin amyloidosis (h-ATTR). However, to optimize the outcome, careful selection of patients is required, since increased mortality compared with that found for nontransplanted historical controls are observed for several groups of h-ATTR patients. We have noted that malnourished patients and patients with a late onset of the disease especially in combination with findings of cardiomyopathy are at risk for an increased mortality and morbidity. Recently detection of different types of amyloid fibrils that appears to be related to the phenotype of the patient may facilitate patient selection for LTx.

Keywords::

• Amyloidosis-hereditary
• cardiomyopathy
• neuropathy
• transplantation-liver

Acknowledgements

The authors also would like to acknowledge the data collected by the international contributors to FAPWTR.

Declaration of Interest: This work was supported by grants from the patient organization FAMY Västerbotten, FAMY/AMYL Norrbotten, the Swedish Heart and Lung Foundation, a Spearhead grant from Västerbotten’s County Council and ALF grants from Umeå University and University Hospital.