Anesth Pain Med.  2020 Oct;15(4):472-477. 10.17085/apm.20059.

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

• Cha S
• Kim J
• Choi SJ
• Kim GS

• KMID: 2508410
• DOI: http://doi.org/10.17085/apm.20059

Abstract

Background
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. Case: 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.
Conclusions
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.

Keyword

Autonomic denervation; Domino liver transplantation; Familial amyloid polyneuropathy; Isoproterenol; Living donor liver transplantation; Transthyretin

Figure

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  Fig. 1. Timeline of the three consecutive operations for the domino liver transplantation. All timelines are relative to the start of the operation for Case 1. I: induction, Gr: graft out, Pre: prehepatic stage, AN: anhepatic stage, Neo: neohepatic stage, Rep reperfusion.

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