Dilated cardiomyopathy
Dilated cardiomyopathy
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DOI：
10.1016/s0140-6736(09)62023-7
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发表时间：
2010-02-27
期刊：
影响因子：
168.9
通讯作者：
Towbin, Jeffrey A.
中科院分区：
文献类型：
--
作者：
Jefferies, John Lynn;Towbin, Jeffrey A.
扩张型心肌病的特征是与收缩功能障碍相关的左心室扩张。可能会出现舒张功能障碍和右心室功能受损。受影响的个体存在左心室衰竭或右心室衰竭或两者兼有的风险。心力衰竭症状可能是运动引起的，也可能是静息时持续存在的。许多患者没有症状。长期接受治疗的患者有时会出现急性失代偿性心力衰竭。其他危及生命的风险包括室性心律失常和房室传导阻滞、晕厥和猝死。 30-48% 的患者存在遗传性，心肌炎等炎症性疾病或药物、酒精或违禁药物的毒性作用也会导致扩张型心肌病。引起扩张型心肌病的基因通常编码细胞骨架和肌节（收缩装置）蛋白，尽管钙稳态的紊乱似乎也很重要。在儿童中，线粒体功能紊乱和代谢异常具有因果作用。治疗的重点是提高心脏效率和减少机械应力。心律失常治疗和猝死预防仍然是治疗的支柱。尽管过去 10 年取得了进展，但成果仍需改进。
Dilated cardiomyopathy is characterised by left ventricular dilation that is associated with systolic dysfunction. Diastolic dysfunction and impaired right ventricular function can develop. Affected individuals are at risk of left or right ventricular failure, or both. Heart failure symptoms can be exercise-induced or persistent at rest. Many patients are asymptomatic. Chronically treated patients sometimes present acutely with decompensated heart failure. Other life-threatening risks are ventricular arrhythmias and atrioventricular block, syncope, and sudden death. Genetic inheritance arises in 30-48% of patients, and inflammatory disorders such as myocarditis or toxic effects from medications, alcohol, or illicit drugs also result in dilated cardiomyopathy. Genes that cause dilated cardiomyopathy generally encode cytoskeletal and sarcomeric (contractile apparatus) proteins, although disturbance of calcium homeostasis also seems to be important. In children, disrupted mitochondrial function and metabolic abnormalities have a causal role. Treatments focus on improvement of cardiac efficiency and reduction of mechanical stress. Arrhythmia therapy and prevention of sudden death continue to be mainstays of treatment. Despite progress over the past 10 years, outcomes need to be improved.