致心律失常性右室发育不良的研究进展

Progress in Arrhythmogenic Right Ventricular Dysplasia

致心律失常性右室发育不良/心肌病是年轻人和运动员疲劳后猝死的主要原因,尽管病因不完全清楚,但基因异常是肯定的。病理学特点主要是右室心肌进行性被纤维脂肪组织替代。临床表现差别也比较大,可表现无症状性猝死,也可表现为发生心悸和(或)右心衰竭或全心衰竭。接近1/3病人12导联心电图出现右室晚激动波,平均信号心电图常表现为晚电位。二维超声心动图、磁共振显像、右室造影均可显示右室形态学异常。治疗主要是治疗和预防致命性心律失常,可根据病人情况采用药物治疗、植入型心律转复除颤器、射频消融治疗等。

Arrhythmogenic right ventricular cardiomyopathy is an important cause of sudden arrhythmic death, often exertional, in young individuals and athletes. Although the aetiology remains partially unknown,genetic abnormalities have been demonstrated. The characteristic pathologic finding is the progressive fibro-fatty replacement of the right ventricular myocardium. The clinical manifestations vary from asymptomatic patients with an episode of sudden cardiac death as first symptom to chronically symptomatic patients with recurrent palpitations and/or right or biventrieular failure. Approximately a third of the patients show the characteristic Epsilon wave in 12-lead ECG which is a use- ful screening test. Signal averaged ECG frequently demonstrates late potentials. The two-dimensional echocardiography, magnetic resonance imaging,computerized tomography and right ventricular cineangiography show morphologic abnormalities in the right ventricle. Therapy is directed to prevent and/or treat malignant ventricular tachyarrhythmias with medications,implantable cardioverter defibrillator and radiofrequency ablation in selected cases.