Baik Soon Koo, Fouad Tamer R, Lee Samuel S
Dept of Medicine, Yonsei University Wonju College of Medicine, Wonju, South Korea.
Orphanet J Rare Dis. 2007 Mar 27;2:15. doi: 10.1186/1750-1172-2-15.
Cirrhotic cardiomyopathy is the term used to describe a constellation of features indicative of abnormal heart structure and function in patients with cirrhosis. These include systolic and diastolic dysfunction, electrophysiological changes, and macroscopic and microscopic structural changes. The prevalence of cirrhotic cardiomyopathy remains unknown at present, mostly because the disease is generally latent and shows itself when the patient is subjected to stress such as exercise, drugs, hemorrhage and surgery. The main clinical features of cirrhotic cardiomyopathy include baseline increased cardiac output, attenuated systolic contraction or diastolic relaxation in response to physiologic, pharmacologic and surgical stress, and electrical conductance abnormalities (prolonged QT interval). In the majority of cases, diastolic dysfunction precedes systolic dysfunction, which tends to manifest only under conditions of stress. Generally, cirrhotic cardiomyopathy with overt severe heart failure is rare. Major stresses on the cardiovascular system such as liver transplantation, infections and insertion of transjugular intrahepatic portosystemic stent-shunts (TIPS) can unmask the presence of cirrhotic cardiomyopathy and thereby convert latent to overt heart failure. Cirrhotic cardiomyopathy may also contribute to the pathogenesis of hepatorenal syndrome. Pathogenic mechanisms of cirrhotic cardiomyopathy are multiple and include abnormal membrane biophysical characteristics, impaired beta-adrenergic receptor signal transduction and increased activity of negative-inotropic pathways mediated by cGMP. Diagnosis and differential diagnosis require a careful assessment of patient history probing for excessive alcohol, physical examination for signs of hypertension such as retinal vascular changes, and appropriate diagnostic tests such as exercise stress electrocardiography, nuclear heart scans and coronary angiography. Current management recommendations include empirical, nonspecific and mainly supportive measures. The exact prognosis remains unclear. The extent of cirrhotic cardiomyopathy generally correlates to the degree of liver insufficiency. Reversibility is possible (either pharmacological or after liver transplantation), but further studies are needed.
肝硬化性心肌病是用于描述肝硬化患者中一系列提示心脏结构和功能异常特征的术语。这些特征包括收缩和舒张功能障碍、电生理变化以及宏观和微观结构变化。目前肝硬化性心肌病的患病率尚不清楚,主要是因为该疾病通常呈潜伏性,在患者受到运动、药物、出血和手术等应激时才会显现出来。肝硬化性心肌病的主要临床特征包括基础心输出量增加、对生理、药理和手术应激时收缩期收缩或舒张期松弛减弱以及电导异常(QT间期延长)。在大多数情况下,舒张功能障碍先于收缩功能障碍,收缩功能障碍往往仅在应激条件下才会表现出来。一般来说,明显严重心力衰竭的肝硬化性心肌病很少见。心血管系统的重大应激,如肝移植、感染和经颈静脉肝内门体分流术(TIPS)置入,可使肝硬化性心肌病的存在显现出来,从而使潜伏性心力衰竭转变为显性心力衰竭。肝硬化性心肌病也可能促成肝肾综合征的发病机制。肝硬化性心肌病的发病机制是多方面的,包括膜生物物理特性异常、β-肾上腺素能受体信号转导受损以及由cGMP介导的负性肌力途径活性增加。诊断和鉴别诊断需要仔细评估患者病史以探查过量饮酒情况、进行体格检查以寻找高血压体征如视网膜血管变化,以及进行适当的诊断测试,如运动应激心电图、核素心脏扫描和冠状动脉造影。目前的管理建议包括经验性、非特异性且主要是支持性的措施。确切的预后仍不清楚。肝硬化性心肌病的程度一般与肝功能不全的程度相关。有可能实现可逆性(无论是通过药物治疗还是肝移植后),但还需要进一步研究。