致心律失常性右室发育不良或心肌病15例的临床特点及疗效

Clinical characteristics and treatment effect of 15 patients with arrhythmogenic right ventricular dysplasia and cardiomyopathy

目的探讨致心律失常性右室发育不良或心肌病(ARVD/C)的临床特点及分析疗效。方法分析2000～2007年诊断为ARVD/C15例入院病例资料,对其临床特点作统计分析,并探讨治疗方法及疗效。结果在ARVD/C15例病例中(7男),年龄为13～61(31±12)岁,首发症状年龄为10～51(28±11)岁;3例有家族史;6例(40%)有晕厥发作史;5例(33%)患者仅有心悸症状;1例常规心电图检查中发现Epsilon波,见于右侧胸导联V2～3,伴有T波倒置;13例(87%)超声心动图结果异常,主要为RV扩大;4例行心脏磁共振(MRI)检查:见右室壁脂肪信号2例,右室壁变薄3例,右心室扩大3例;有症状的室性心律失常患者接受胺碘酮、β阻滞剂或采用其他抗心律失常药物治疗,但47%的患者(7/15)应用抗心律失常药物治疗无效,3例患者接受射频消融治疗,其中有1例患者出现室性心动过速复发。4例患者植入植入式心脏自动复律除颤器(ICD),其中1例因多次自动除颤,电池耗竭,而更换ICD。结论ARVD/C以室性心律失常为主要表现,诊断依靠家族史、晕厥发作史、ECG、超声心动图、MRI。抗心律失常药物的疗效较差,射频消融或植入ICD可治疗致命性心律失常,减少猝死的发生。

AIM： To analyze the clinical characteristics and treatment effect of arrhythmogenic right ventricular dysplasia and cardiomyopathy （ARVD/C）. METHODS： Clinical features were reevaluated in 15 patients diagnosed with ARVD/C from 2000 to 2007 in the Department of Cardiology, Nanfang Hospital. Statistical analysis was carried out and treatment effect was evaluated. RESULTS： In a cohort of 15 patients （7 males） , mean age at the time of hospitalization was （31 ± 12） years and mean age at initial presentation was （28± 11 ） years. Three patients had a significant family history. Six patients （40%） had a history of syncope. Five cases （33%） had slight palpitations. Only one patient had epsilon waves in right preeordial leads （V2-3 ） , with T-wave inversions. Abnormal echoeardiography was found in 13 patients （87%）, mainly right ventricular （RV） dilation. Four patients received magnetic resonance imaging （MRI） and fat signal intensity of RV wall was found in two cases, thinning of RV wall in three cases, and right ventricular hypertrophy in three cases. For symptomatic patients, ventricular arrhythmias were treated with amiodarone, B-blocker or other antiarrhythmic therapies. Antiarrhythmic drugs were ineffective in 47% （7/15） patients and three patients were therefore treated by ablations. Nevertheless, ventricular tachycardia recurred in one patient. Four patients had implanted cardiac defibrillation （ICD）.However, one of them had ICD twice because the battery was exhausted. CONCLUSION： ARVD/C is characterized by ventricular arrhythmias. Diagnosis of ARVD/C may be accomplished by family history, history of syncope, ECG, echocardiography and cardiac MRI. Antiarrhythmic drugs are not very effective. Ablations or ICD can treat ventricular arrhythmias to reduce the incidence of sudden cardiac death.