Managing post-reperfusion syndrome in domino liver transplantation for familial amyloidotic polyneuropathy

Abstract

Familial amyloidotic polyneuropathy is a rare genetic disorder caused by transthyretin mutations, leading to multi-organ dysfunction, with a significant impact on the nervous and cardiovascular systems. Domino liver transplantation is a unique strategy which increases donor organ availability by transplanting the explanted liver from a patient with familial amyloidotic polyneuropathy into another patient. This report describes a 37-year-old patient with familial amyloidotic polyneuropathy who underwent a living donor liver transplantation as part of a domino liver transplantation, emphasising the lessons learnt about the role of isoprenaline for managing the post-reperfusion syndrome and the considerations regarding prophylactic pacemaker insertion. She developed severe bradycardia following anaesthesia induction and again during graft reperfusion, which was refractory to atropine but successfully managed with isoprenaline. On postoperative day 20, she experienced severe bradycardia which was unresponsive to medical management, necessitating a temporary pacemaker, followed by a permanent pacemaker on postoperative day 26. This case highlights the role of isoprenaline as a preferred agent for managing bradyarrhythmia and conduction blocks during liver graft reperfusion. Additionally, it highlights the potential need for prophylactic pacemaker insertion in select patients with subclinical conduction abnormalities, as stressors, such as liver transplantation, can unmask life-threatening arrhythmias.