Kharoubi Mounira, Roche Fréderic, Bézard Mélanie, Hupin David, Silva Sidney, Oghina Silvia, Chalard Coraline, Zaroui Amira, Galat Arnault, Guendouz Soulef, Canoui-Poitrine Florence, Hittinger Luc, Teiger Emmanuel, Lefaucheur Jean-Pascal, Damy Thibaud
Cardiology Department, AP-HP (Assistance Publique-Hôpitaux de Paris), Henri Mondor University Hospital, 51 Avenue du Marechal de Lattre de Tassigny, Créteil, F-94010, France.
French Referral Centre for Cardiac Amyloidosis, Cardiogen Network, Créteil, France.
ESC Heart Fail. 2021 Apr;8(2):1656-1665. doi: 10.1002/ehf2.13131. Epub 2020 Dec 22.
The prevalence of autonomic neuropathy (AN) is high in patients with hereditary transthyretin amyloidosis but remains unknown in transthyretin wild-type cardiac amyloidosis (ATTRwt-CA). This study aimed to determine the prevalence of AN in patients with ATTRwt-CA using Sudoscan®, a non-invasive method used to provide evidence of AN in clinical practice and based on measurement of electrochemical skin conductance at the hands and feet (fESC).
A series of 62 non-diabetic patients with ATTRwt-CA was prospectively included over 2 years and compared with healthy elderly subjects, matched by age, gender, and body mass index. The presence of AN was defined as electrochemical skin conductance at the hands <60 μS and/or fESC <70 μS, and conductances were analysed according to clinical, biological, and echocardiographic data. Mean fESC was significantly lower in patients with ATTRwt-CA compared with elderly controls: 68.3 (64.1-72.5) vs. 76.9 (75.6-78.1) μS (P < 0.0001), respectively. Prevalence of fESC <70 μS was higher in ATTRwt-CA patients than in controls: 48.4% vs. 19.9%, P < 0.05. Univariate analysis showed that fESC, N-terminal pro-B-type natriuretic peptide, creatinine plasma levels, and echocardiographic global longitudinal strain were associated with decompensated cardiac failure and death. Multivariate analysis revealed that fESC was an independent prognostic factor, and Kaplan-Meier estimator evidenced a greater occurrence of cardiac decompensation and death in patients with fESC <70 μS, P = 0.046.
Reduced fESC was observed in almost 50% of patients with ATTRwt-CA and was associated with a worse prognosis. Sudoscan® could easily be used to screen ATTRwt-CA patients for the presence of AN and identify patients at higher risk for a poor outcome.
遗传性转甲状腺素蛋白淀粉样变性患者中自主神经病变（AN）的患病率较高，但在转甲状腺素蛋白野生型心脏淀粉样变性（ATTRwt-CA）患者中仍不清楚。本研究旨在使用Sudoscan®确定ATTRwt-CA患者中AN的患病率，Sudoscan®是一种非侵入性方法，用于在临床实践中提供AN的证据，基于对手和脚的电化学皮肤电导（fESC）的测量。
在2年期间前瞻性纳入了一系列62例非糖尿病ATTRwt-CA患者，并与年龄、性别和体重指数相匹配的健康老年受试者进行比较。AN的存在定义为手部电化学皮肤电导<60μS和/或fESC<70μS，并根据临床、生物学和超声心动图数据分析电导。与老年对照组相比，ATTRwt-CA患者的平均fESC显著降低：分别为68.3（64.1-72.5）与76.9（75.6-78.1）μS（P<0.0001）。ATTRwt-CA患者中fESC<70μS的患病率高于对照组：48.4%对19.9%，P<0.05。单因素分析表明，fESC、N末端前B型利钠肽、肌酐血浆水平和超声心动图整体纵向应变与失代偿性心力衰竭和死亡相关。多因素分析显示，fESC是一个独立的预后因素，Kaplan-Meier估计表明，fESC<70μS的患者心脏失代偿和死亡的发生率更高，P=0.046。
在近50%的ATTRwt-CA患者中观察到fESC降低，并且与较差的预后相关。Sudoscan®可轻松用于筛查ATTRwt-CA患者是否存在AN，并识别预后不良风险较高的患者。
