a Cardiology department , Bichat Claude Bernard Hospital, AP-HP, Université Paris Diderot , Paris , France.
b Hepato-Biliary Center, Paul Brousse hospital, AP-HP, UMR-S 785, Univ. Paris-Sud , Villejuif , France.
Amyloid. 2018 Dec;25(4):253-260. doi: 10.1080/13506129.2018.1550061. Epub 2019 Jan 11.
Hereditary transthyretin amyloidosis (ATTR) is a multisystemic disease involving mainly the peripheral nervous system and the heart. Liver transplantation (LT) is the reference treatment for ATTR neuropathy and preoperative detection of high risk patients is crucial. We aimed to document the causes of death of ATTR patients after LT, their temporal trends, and to evaluate whether the available preoperative tools that predict the risk of death after LT for hereditary ATTR amyloidosis matched with these trends.
A retrospective longitudinal cohort study was performed on 215 consecutive ATTR patients who underwent LT between January 1993 and January 2011. Each patient's death cause and timing were classified.
Over a median follow up of 5.9 years, 84 patients died. The rate of death was higher in the first year following LT than thereafter (13.0 vs. 4.3 ± 1.8%/year; p = .004). Cardiac events ranked as the leading cause of death (C: 38%), followed by infections (I: 24%), graft complications (G: 17%), end stage amyloidosis, stroke and others (ASO: 7% each). Deaths due to graft complications and infections (GI) occurred earlier than those due to end stage amyloidosis and stroke. Death prediction was less accurate for GI-related mortality than for other causes, which blunted the accuracy of the early-term risk prediction scores. Conclusions In ATTR amyloidosis, cardiac events were the leading cause of death after liver transplantation. Close preoperative evaluation allowed for accurate mid-term prediction of mortality, but the high rate of graft complications and infections blunted the early-term risk prediction.
遗传性转甲状腺素蛋白淀粉样变性（ATTR）是一种多系统疾病，主要累及周围神经系统和心脏。肝移植（LT）是 ATTR 神经病的首选治疗方法，术前检测高危患者至关重要。我们旨在记录 LT 后 ATTR 患者的死亡原因、时间趋势，并评估术前预测 LT 后遗传性 ATTR 淀粉样变性死亡风险的工具是否与这些趋势相匹配。
对 1993 年 1 月至 2011 年 1 月期间接受 LT 的 215 例连续 ATTR 患者进行回顾性纵向队列研究。对每位患者的死亡原因和时间进行分类。
在中位随访 5.9 年后，84 例患者死亡。LT 后第一年的死亡率高于此后（13.0 比 4.3±1.8%/年；p=0.004）。心脏事件是导致死亡的主要原因（C：38%），其次是感染（I：24%）、移植物并发症（G：17%）、终末期淀粉样变性、中风和其他（ASO：7%）。移植物并发症和感染（GI）导致的死亡早于终末期淀粉样变性和中风。GI 相关死亡率的预测准确性低于其他原因，这削弱了早期风险预测评分的准确性。结论：在 ATTR 淀粉样变性中，心脏事件是 LT 后死亡的主要原因。术前仔细评估可准确预测中期死亡率，但移植物并发症和感染的高发生率削弱了早期风险预测。
