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.