Liver transplantation for hereditary transthyretin amyloidosis

Transthyretin (TTR) amyloidosis is the most common form of hereditary amylo idosis. It is a systemic amyloidosis caused by an amyloidogenic variant TTR (ATTR), of which the methionine for valine at position 30 (ATTR Val30Met) gives rise to a fatal neuropathic amyloidosis, Because more than 95% of TTR is produced by the liver, a liver transplantation should abolish the liver 's production of amyloidogenic mutant TTR and thereby halt amyloid formatio n, The first liver transplantation for hereditary TTR amyloidosis was perfo rmed in Sweden in 1990 on a patient with ATTR Val30Met amyloidosis, and the result was encouraging. Today, liver transplantation for TTR amyloidosis i s an established treatment. However, the disease is rarely seen except in a few endemic areas; therefore, most transplantation centers only receive a few cases, Because the disease phenotype varies with different TTR mutation s and variability is even encountered for the same mutation, an evaluation of patients for transplantation must include an investigation of all organs that may be affected by the disease and may impact on the morbidity and mo rtality of the procedure. The aim of this review is to present the results of liver transplantation for TTR amyloidosis and give recommendations for p atient evaluation and selection based on the literature and our experience with the disease.