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肝硬化性心肌病

Cirrhotic cardiomyopathy.

作者信息

Ruiz-del-Árbol Luis, Serradilla Regina

机构信息

Luis Ruiz-del-Árbol, Regina Serradilla, Hepatic Hemodynamic Unit, Gastroenterology Department, Hospital Ramón y Cajal, University of Alcalá, 28034 Madrid, Spain.

出版信息

World J Gastroenterol. 2015 Nov 7;21(41):11502-21. doi: 10.3748/wjg.v21.i41.11502.

Abstract

During the course of cirrhosis, there is a progressive deterioration of cardiac function manifested by the disappearance of the hyperdynamic circulation due to a failure in heart function with decreased cardiac output. This is due to a deterioration in inotropic and chronotropic function which takes place in parallel with a diastolic dysfunction and cardiac hypertrophy in the absence of other known cardiac disease. Other findings of this specific cardiomyopathy include impaired contractile responsiveness to stress stimuli and electrophysiological abnormalities with prolonged QT interval. The pathogenic mechanisms of cirrhotic cardiomyopathy include impairment of the b-adrenergic receptor signalling, abnormal cardiomyocyte membrane lipid composition and biophysical properties, ion channel defects and overactivity of humoral cardiodepressant factors. Cirrhotic cardiomyopathy may be difficult to determine due to the lack of a specific diagnosis test. However, an echocardiogram allows the detection of the diastolic dysfunction and the E/e' ratio may be used in the follow-up progression of the illness. Cirrhotic cardiomyopathy plays an important role in the pathogenesis of the impairment of effective arterial blood volume and correlates with the degree of liver failure. A clinical consequence of cardiac dysfunction is an inadequate cardiac response in the setting of vascular stress that may result in renal hypoperfusion leading to renal failure. The prognosis is difficult to establish but the severity of diastolic dysfunction may be a marker of mortality risk. Treatment is non-specific and liver transplantation may normalize the cardiac function.

摘要

在肝硬化病程中,心脏功能会逐渐恶化,表现为由于心功能衰竭导致心输出量减少,进而高动力循环消失。这是由于在没有其他已知心脏疾病的情况下,心肌收缩力和心率调节功能恶化,同时伴有舒张功能障碍和心肌肥厚。这种特定心肌病的其他表现包括对应激刺激的收缩反应受损以及QT间期延长的电生理异常。肝硬化性心肌病的发病机制包括β-肾上腺素能受体信号传导受损、心肌细胞膜脂质成分和生物物理特性异常、离子通道缺陷以及体液性心脏抑制因子活性过高。由于缺乏特异性诊断测试,肝硬化性心肌病可能难以确诊。然而,超声心动图可检测到舒张功能障碍,E/e'比值可用于疾病进展的随访。肝硬化性心肌病在有效动脉血容量受损的发病机制中起重要作用,并与肝衰竭程度相关。心脏功能障碍的一个临床后果是在血管应激情况下心脏反应不足,这可能导致肾灌注不足,进而引发肾衰竭。预后难以确定,但舒张功能障碍的严重程度可能是死亡风险的一个指标。治疗无特异性,肝移植可能使心脏功能恢复正常。

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