Dilemmas and Challenges in the Anesthetic Management of Liver Transplantation for Transthyretin Amyloidosis in the Asian Context: A Case Report.

Transthyretin (TTR) amyloidosis is a progressive, debilitating, and eventually fatal disease that is under-recognized and underdiagnosed in Asian patients. Liver transplantation is performed in patients with hereditary transthyretin amyloidosis to remove the source of abnormal transthyretin production to slow disease progression. There is uncertainty about the risk-benefit ratio due to the less favourable five-year survival of liver transplantation for Asian patients with non-Val30Met transthyretin amyloidosis, as the heterogeneity of non-Val30Met mutations and the potential for disease progression despite transplantation can make transplant outcomes unpredictable. Additionally, limited access and the prohibitive costs of disease-modifying therapies may influence the decision-making process with regard to liver transplantation. We present a case of a 57-year-old male with Ala117Ser TTR amyloidosis and clinical manifestations of polyneuropathy, cardiac amyloidosis, and autonomic neuropathy. He received a living-related liver transplant. We describe the dilemmas and challenges in the anesthetic planning and management of this patient. Along with limited scientific data on liver transplantation for non-Val30Met TTR amyloidosis patients, we faced challenges in the management of intraoperative tachyarrhythmias, which eventually necessitated the use of a relatively contraindicated drug, verapamil, to achieve rate control. The lack of specific data meant that we had to base some decisions on practical wisdom.