Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
Lancet. 2010 Feb 27;375(9716):752-62. doi: 10.1016/S0140-6736(09)62023-7.
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.
扩张型心肌病的特征为左心室扩张，伴有收缩功能障碍。舒张功能障碍和右心室功能障碍可随之发生。受累个体有发生左心室或右心室衰竭的风险，或两者皆有。心力衰竭症状可由运动引起，也可持续存在于休息时。许多患者无症状。慢性治疗的患者有时会因心力衰竭失代偿而急性发作。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%的患者有遗传继承，炎症性疾病如心肌炎或药物、酒精或非法药物的毒性作用也可导致扩张型心肌病。导致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，但钙稳态的紊乱似乎也很重要。在儿童中，线粒体功能障碍和代谢异常起因果作用。治疗重点在于提高心脏效率和降低机械应激。心律失常治疗和预防猝死仍然是治疗的主要方法。尽管在过去 10 年中取得了进展，但仍需要改善预后。
