Department of Cardiology, Angiology, and Respiratory Medicine, University Hospital Heidelberg, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany.
Department of Psychology, University of Heidelberg, Heidelberg, Germany.
Clin Res Cardiol. 2018 Feb;107(2):158-169. doi: 10.1007/s00392-017-1167-1. Epub 2017 Sep 27.
To analyze clinical predictors of mortality in wild-type transthyretin amyloidosis (wt-ATTR).
In total, 191 patients (73.8 ± 0.5 years; 176 males, 15 females) with histologically proven wt-ATTR amyloidosis and genetic exclusion of a transthyretin gene variant were included. Comprehensive clinical characteristics, ECG, biomarkers, and echocardiography were analyzed retrospectively. Strain analyses were performed offline using TomTec Imaging Systems, Germany. Univariable and multivariable analyses predicting all-cause mortality were carried out.
Patients presented with significant heart failure (NYHA 2.5 ± 0.8; NT-proBNP 3644 (4981) pg/ml; LV ejection fraction 45.8 ± 15.0%). LogNT-proBNP correlated with indicators of disease severity. Similar results were obtained for basal and midventricular, but not apical longitudinal strain. During median follow-up of 26.2 ± 1.7 months 46 (25.5%) patients died (40 males, 23%; six females, 40%). In female patients 1-/2-year survival was lower [92.9/67.7%; median survival 30.6 (21.1-40.1) months] when compared to male patients [96.5%/86.6%; median survival 63.9 (45.8-82.0) months]. Parameters associated with survival were NT-proBNP, NYHA class, heart rate, midventricular longitudinal strain, mitral annular plane systolic excursion (MAPSE), Karnofsky Index, systolic blood pressure, estimated glomerular filtration rate. Multivariable analysis revealed MAPSE and NT-proBNP as independent predictors of mortality in the whole cohort and midventricular strain in the subgroup of patients in sinus rhythm.
No sex-specific bias was observed between male and female patients with wt-ATTR regarding age at onset and morphological characteristics. Multivariable analysis revealed MAPSE and NT-proBNP as independent predictors of survival in the whole cohort, whereas midventricular longitudinal strain was the only independent predictor in patients in sinus rhythm.
分析野生型转甲状腺素蛋白淀粉样变（wt-ATTR）患者的死亡临床预测因子。
共纳入 191 例组织学证实的 wt-ATTR 淀粉样变且排除转甲状腺素基因变异的患者。回顾性分析了全面的临床特征、心电图、生物标志物和超声心动图。使用德国 TomTec 成像系统离线进行应变分析。进行单变量和多变量分析以预测全因死亡率。
患者表现出明显的心衰（NYHA 2.5±0.8；NT-proBNP 3644（4981）pg/ml；左心室射血分数 45.8±15.0%）。LogNT-proBNP 与疾病严重程度的指标相关。基底和中层心室的应变结果相似，但心尖纵向应变结果不同。在 26.2±1.7 个月的中位随访期间，46 例（25.5%）患者死亡（40 例男性，23%；6 例女性，40%）。与男性患者相比，女性患者的 1 年和 2 年生存率较低[92.9%/67.7%；中位生存时间 30.6（21.1-40.1）个月]。与生存相关的参数包括 NT-proBNP、NYHA 分级、心率、中层心室纵向应变、二尖瓣环平面收缩期位移（MAPSE）、卡诺夫斯基指数、收缩压、估算肾小球滤过率。多变量分析显示，MAPSE 和 NT-proBNP 是整个队列死亡率的独立预测因子，而在窦性心律患者亚组中，中层心室应变是唯一的独立预测因子。
在 wt-ATTR 患者中，男性和女性在发病年龄和形态特征方面没有性别特异性差异。多变量分析显示，MAPSE 和 NT-proBNP 是整个队列生存率的独立预测因子，而在窦性心律患者中，中层心室纵向应变是唯一的独立预测因子。
