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心脏淀粉样变性的血流动力学特征及其预后相关性

Hemodynamic Profiles and Their Prognostic Relevance in Cardiac Amyloidosis.

作者信息

Duca Franz, Snidat Amir, Binder Christina, Rettl René, Dachs Theresa-Marie, Seirer Benjamin, Camuz-Ligios Luciana, Dusik Fabian, Capelle Christophe Denis Josef, Hong Qin, Agis Hermine, Kain Renate, Mascherbauer Julia, Hengstenberg Christian, Badr Eslam Roza, Bonderman Diana

机构信息

Department of Internal Medicine II, Department of Cardiology, Medical University of Vienna, 1090 Vienna, Austria.

Department of Internal Medicine I, Department of Oncology, Medical University of Vienna, 1090 Vienna, Austria.

出版信息

J Clin Med. 2020 Apr 11;9(4):1093. doi: 10.3390/jcm9041093.

Abstract

This study sought to characterize cardiac amyloidosis (CA) patients with respect to hemodynamic parameters and asses their prognostic impact in different CA cohorts. Intracardiac and pulmonary arterial pressures (PAPs) are among the strongest predictors of outcomes in patients with heart failure (HF). Despite that, the hemodynamic profiles of patients with CA and their relation to prognosis have rarely been investigated. Invasive hemodynamic, clinical, and laboratory assessment, as well as cardiac magnetic resonance imaging were performed in our CA cohort. A total of 61 patients, 35 (57.4%) with wild-type transthyretin amyloidosis (ATTRwt) and 26 (42.6%) with light-chain amyloidosis (AL) were enrolled. ATTRwt patients had lower N-terminal prohormone of brain natriuretic peptide values and were less frequently in New York Heart Association class ≥ III. Intracardiac and PAPs were elevated, but hemodynamic parameters did not differ between CA groups. Whereas in ATTRwt, the median mean PAP (hazard ratio (HR): 1.130, = 0.040) and pulmonary vascular resistance (HR: 1.010, = 0.046) were independent predictors of outcome, no hemodynamic parameter was associated with outcome in the AL group. Cardiac ATTRwt and AL patients feature elevated intracardiac and PAPs and show similar hemodynamic profiles. However, hemodynamic parameters are of greater prognostic relevance in ATTRwt, potentially providing a new therapeutic target.

摘要

本研究旨在根据血流动力学参数对心脏淀粉样变性(CA)患者进行特征描述,并评估其在不同CA队列中的预后影响。心内压和肺动脉压(PAPs)是心力衰竭(HF)患者预后的最强预测因素之一。尽管如此,CA患者的血流动力学特征及其与预后的关系却鲜有研究。我们对CA队列患者进行了有创血流动力学、临床和实验室评估以及心脏磁共振成像检查。共纳入61例患者,其中35例(57.4%)为野生型转甲状腺素蛋白淀粉样变性(ATTRwt),26例(42.6%)为轻链淀粉样变性(AL)。ATTRwt患者的N末端脑钠肽前体值较低,纽约心脏协会心功能分级≥III级的情况也较少见。心内压和PAPs升高,但CA组之间的血流动力学参数并无差异。在ATTRwt患者中,平均PAP中位数(风险比(HR):1.130,P = 0.040)和肺血管阻力(HR:1.010,P = 0.046)是预后的独立预测因素,而在AL组中,没有血流动力学参数与预后相关。心脏ATTRwt和AL患者的心内压和PAPs升高,血流动力学特征相似。然而,血流动力学参数在ATTRwt中具有更大的预后相关性,可能提供一个新的治疗靶点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2df7/7230541/2a6a73f3a390/jcm-09-01093-g001.jpg

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