Sindwani G, Ronanki S L, Yadav A, Dhingra U, Tempe D, Pamecha V, Mohapatra N
Department of Anaesthesia and Intensive Care Institute of Liver and Biliary Sciences New Delhi India.
Department of Hepato-Pancreato-Biliary Surgery and Liver Transplantation Institute of Liver and Biliary Sciences New Delhi India.
Anaesth Rep. 2025 Mar 20;13(1):e70006. doi: 10.1002/anr3.70006. eCollection 2025 Jan-Jun.
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.
家族性淀粉样多神经病是一种由转甲状腺素蛋白突变引起的罕见遗传性疾病，可导致多器官功能障碍，对神经和心血管系统有重大影响。多米诺肝移植是一种独特的策略，通过将家族性淀粉样多神经病患者的切除肝脏移植给另一名患者来增加供体器官的可用性。本报告描述了一名37岁的家族性淀粉样多神经病患者，他接受了活体供肝移植作为多米诺肝移植的一部分，强调了关于异丙肾上腺素在处理再灌注综合征中的作用以及预防性植入起搏器的考虑所学到的经验教训。她在麻醉诱导后以及移植肝再灌注期间再次出现严重心动过缓，对阿托品无效，但用异丙肾上腺素成功处理。术后第20天，她出现严重心动过缓，药物治疗无效，需要临时起搏器，术后第26天植入永久起搏器。该病例突出了异丙肾上腺素作为肝移植再灌注期间处理缓慢性心律失常和传导阻滞的首选药物的作用。此外，它还突出了在某些有亚临床传导异常的患者中预防性植入起搏器的潜在必要性，因为诸如肝移植等应激源可能会引发危及生命的心律失常。
