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Heart：von Willebrand因子在先天性心脏病成人中的预后价值

2020-03-19 MedSci原创 MedSci原创

von Willebrand因子，先天性心脏病成人,预后价值
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高vWF:Ag可能反映了RHF的严重程度和相关的肝功能障碍，对ACHD的全因死亡具有较强的预后价值。因此，vWF:Ag可能是监测伴有RHF的ACHD优良的指标。

血管性假血友病因子(von Willebrand factor，vWF)对心力衰竭(HF)患者和肝病患者具有预后价值。右心衰(RHF)引起的肝脏充血是先天性心脏病成人(ACHD)主要的临床病理生理表现之一。近日，心脏病领域权威杂志Heart上发表了一篇研究文章，该研究的目的是阐明血浆vWF抗原(vWF:Ag)水平在ACHD中的预后价值。

研究人员对382例(20例未修复的青紫性ACHD，17例Fontan患者，190例双室修复后的ACHD)患者的vWF:Ag(%)进行测定，并与患者的临床资料及预后进行比较。

血浆vWF:Ag水平为130±53(正常范围:55%~190%)，48例(13%)患者vWF:Ag水平较高(≥190%)。高龄、Fontan循环、较高的中心静脉压、较低的动脉血氧饱和度和较低的血浆白蛋白水平与较高的(vWF:Ag)对数独立相关(p<0.05-0.0001)。在2.4±1.4年的随访中，15例患者死亡。较高的(vWF:Ag)对数可预测全因死亡率(HR为1.63/0.1，95%CI为1.40~1.96, p<0.0001)。具体而言，高vWF:Ag(≥165%)患者的全因死亡风险显著增高(HR为56.4, 95%CI为11.4~1020, p<0.0001)，且该预后值与血浆中脑型利钠肽水平无关。

由此可见，高vWF:Ag可能反映了RHF的严重程度和相关的肝功能障碍，对ACHD的全因死亡具有较强的预后价值。因此，vWF:Ag可能是监测伴有RHF的ACHD优良的指标。

原始出处：

Hideo Ohuchi.et al.Prognostic value of von Willebrand factor in adult patients with congenital heart disease.heart.2020.http://dx.doi.org/10.1136/heartjnl-2019-316007

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    2020-03-21 zhaojie88

    #ART#

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    2020-03-21 huirong

    #先天性#

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    2020-03-21 paperyu

    #预后价值#

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    2020-03-21 slcumt

    #HEART#

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