Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden.
Department of Transplantation Surgery, Karolinska University Hospital, Huddinge, Sweden.
Transplantation. 2018 Feb;102(2):e59-e66. doi: 10.1097/TP.0000000000001975.
Central nervous system (CNS) complications are increasingly noted in liver transplanted (LTx) hereditary transthyretin amyloid (ATTRm) amyloidosis patients; this suggests that the increased survival allows for intracranial ATTRm formation from brain synthesized mutant TTR. However, atrial fibrillation (AF), a recognised risk factor for ischemic CNS complications, is also observed after LTx. The aim of the study was to investigate the occurrence of CNS complications and AF in LTx ATTRm amyloidosis patients.
The medical records of all LTx ATTRm amyloidosis patients in the county of Västerbotten, Sweden, were investigated for information on CNS complications, AF, anticoagulation (AC) therapy, hypertension, cardiac ischemic disease, hypertrophy, and neurological status.
Sixty-three patients that had survived for 3 years or longer after LTx were included in the analysis. Twenty-five patients had developed 1 or more CNS complications at a median of 21 years after onset of disease. AF was noted in 21 patients (median time to diagnosis 24 years). Cerebrovascular events (CVE) developed in 17 (median time to event 21 years). CVEs occurred significantly more often in patients with AF (P < 0.002). AC therapy significantly reduced CVEs, including bleeding in patients with AF (P = 0.04). Multivariate analysis identified AF as the only remaining regressor with a significant impact on CVE (hazard ratio, 3.8; 95% confidence interval 1.1-9.5; P = 0.029).
AF is an important risk factor for CVE in LTx ATTRm amyloidosis patients, and AC therapy should be considered. However, the increased bleeding risk with AC therapy in patients with intracranial amyloidosis should be acknowledged.
中枢神经系统（CNS）并发症在接受肝移植（LTx）遗传性转甲状腺素蛋白（ATTRm）淀粉样变（ATTRm）淀粉样变性患者中越来越常见；这表明，存活率的提高允许脑内 ATTRm 从大脑合成的突变 TTR 形成。然而，心房颤动（AF）是缺血性 CNS 并发症的一个公认危险因素，也在 LTx 后观察到。本研究旨在探讨 LTx ATTRm 淀粉样变性患者 CNS 并发症和 AF 的发生情况。
对瑞典韦斯特博滕县所有接受 LTx 的 ATTRm 淀粉样变性患者的病历进行了调查，以获取 CNS 并发症、AF、抗凝（AC）治疗、高血压、缺血性心脏病、肥大和神经状态的信息。
63 例患者在 LTx 后存活 3 年或更长时间，包括在分析中。25 例患者在疾病发病后 21 年内出现 1 种或多种 CNS 并发症。21 例患者（诊断中位时间 24 年）发现 AF。17 例患者发生脑血管事件（CVE）（事件中位时间 21 年）。AF 患者发生 CVE 的频率明显更高（P < 0.002）。AC 治疗显著降低了包括 AF 患者出血在内的 CVE（P = 0.04）。多变量分析确定 AF 是影响 CVE 的唯一具有显著影响的回归因子（危险比，3.8；95%置信区间，1.1-9.5；P = 0.029）。
AF 是 LTx ATTRm 淀粉样变性患者 CVE 的重要危险因素，应考虑 AC 治疗。然而，应认识到颅内淀粉样变性患者 AC 治疗的出血风险增加。
