Domino living donor liver transplantation of familial amyloid polyneuropathy patient - A case report

Familial amyloid polyneuropathy (FAP) is caused by mutation in a gene transcribing transport protein produced mainly by the liver. Liver transplantation is required to stop FAP progression, but the pathology causes anesthetic management challenges. We report a case of domino living donor liver trans...

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Bibliographic Details
Published in:Anesthesia and pain medicine 2020, 15(4), , pp.472-477
Main Authors: Cha, Sungrok, Kim, Jiwon, Choi, Soo Joo, Kim, Gaab Soo
Format: Article
Language:English
Subjects:
autonomic denervation
domino liver transplantation
familial amyloid polyneuropathy
isoproterenol
liver transplantation
living donor
Transplantation Anesthesia
transthyretin
마취과학
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Summary:Familial amyloid polyneuropathy (FAP) is caused by mutation in a gene transcribing transport protein produced mainly by the liver. Liver transplantation is required to stop FAP progression, but the pathology causes anesthetic management challenges. We report a case of domino living donor liver transplantation in an FAP patient. No intraoperative events occurred; however, during postoperative day 1 in the intensive care unit (ICU), the FAP patient underwent multiple cardiopulmonary resuscitation (CPR) sessions due to pulseless electrical activity following a sudden drop in blood pressure and ventricular tachycardia. Despite ICU management, the patient died after the third CPR session. Various anesthetic management techniques should be considered for FAP patients. Anesthetic management was carefully assessed with the use of isoflurane, isoproterenol, and an external patch. The cause of deterioration in the ICU is unclear, but further investigation is needed to prevent and better manage postoperative morbidity and mortality.
ISSN:1975-5171
2383-7977
DOI:10.17085/apm.20059