Milner James, Teixeira Rafaela Nicolau, Marinho Ana Vera, Silva Nuno, Calretas Suzana, Ferrão José, Furtado Emanuel, Telo Maria João, Ventura Miguel, Cristóvão João, Elvas Luís, Pêgo Guilherme Mariano, António Natália
Cardiology Department, Coimbra Hospital and University Centre, Coimbra, Portugal.
Faculty of Medicine, University of Coimbra, Coimbra, Portugal.
J Interv Card Electrophysiol. 2019 Aug;55(2):207-211. doi: 10.1007/s10840-019-00517-y. Epub 2019 Mar 9.
Despite the important role of cardiac pacing in preventing syncope and sudden cardiac death in familial amyloid polyneuropathy (FAP), we lack clear guidelines as to the ideal timing and indications for permanent pacemaker implantation.
The purpose of this study was to evaluate the ideal timing for pacemaker implantation in FAP patients submitted to liver transplantation.
Retrospective study of 258 FAP patients submitted to liver transplantation between 1992 and 2012. Comparison of three groups: (A) patients without pacemaker (N = 122); (B) patients submitted to pacemaker implantation after liver transplantation, with documented conduction disorders (N = 73); and (C) patients submitted to "prophylactic" pacemaker implantation before transplantation, (N = 73). Patients were followed up for 12.2 ± 6.7 years.
The majority of patients (57%) were referred for pacemaker implantation, which occurred before liver transplantation in 50% of cases. Patients who required pacemaker after transplantation presented significantly higher Machado-Joseph Score during pre-transplant evaluation than those who did not require pacemaker (24 ± 10 vs 20 ± 10, p = .025), and also exhibited higher levels of hepatic cytolysis enzymes and hyperbilirubinemia. The most common indication for permanent pacemaker was first degree atrioventricular block, with a mean time between transplantation and pacemaker implantation of 8.7 ± 4.2 years. During long-term follow-up, all-cause mortality was 27% and was lowest in the group submitted to pacemaker implantation only after liver transplantation (p = 0.002).
The majority of FAP patients submitted to liver transplantation will need a pacemaker at some time of follow-up. However, it seems that there is no benefit in "prophylactic" cardiac pacing before liver transplantation.
尽管心脏起搏在预防家族性淀粉样多神经病（FAP）患者晕厥和心源性猝死方面发挥着重要作用，但对于永久性起搏器植入的理想时机和适应证，我们仍缺乏明确的指南。
本研究旨在评估接受肝移植的FAP患者起搏器植入的理想时机。
对1992年至2012年间接受肝移植的258例FAP患者进行回顾性研究。比较三组患者：（A）未植入起搏器的患者（N = 122）；（B）肝移植后植入起搏器且有传导障碍记录的患者（N = 73）；（C）移植前接受“预防性”起搏器植入的患者（N = 73）。对患者进行了12.2±6.7年的随访。
大多数患者（57%）被推荐植入起搏器，其中50%的病例在肝移植前植入。移植后需要起搏器的患者在移植前评估时的马查多-约瑟夫评分显著高于不需要起搏器的患者（24±10 vs 20±10，p = 0.025），并且还表现出更高水平的肝细胞溶解酶和高胆红素血症。永久性起搏器植入的最常见适应证是一度房室传导阻滞，移植与起搏器植入之间的平均时间为8.7±4.2年。在长期随访中，全因死亡率为27%，仅在肝移植后植入起搏器的组中最低（p = 0.002）。
大多数接受肝移植的FAP患者在随访的某个时间需要起搏器。然而，肝移植前进行“预防性”心脏起搏似乎没有益处。
