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目的对致心律失常性右室心肌病(ARVC)作回顾性临床分析。方法根据1994年欧洲心脏病协会的诊断标准选择确诊 ARVC 患者31例,分析临床表现、心电学特征及治疗方式,随访疗效。结果 31例中男性27例,女性4例,首次确诊年龄为19～58(34.7±9.4)岁。28例(90.3%)临床症状为心悸、头晕,13例(41.9%)具有晕厥史,3例(9.7%)以晕厥为首发症状,1例有猝死家族史。超声心动图和(或)核磁共振检查,29例表现为右心室扩大,其中2例合并左心室扩大。静息心电图表现为不同程度的 T 波倒置,主要发生在胸前导联;17例(54.8%)可见 epsilon(ε)波;26例(83.9%)平均 QRS 时程≥110 ms,右胸导联 QRS 时程大于左胸导联,平均 QRS 波在 V_(1～3)和 V_(4～5)导联分别为(120.8±13.7)ms和(99.4±13.7)ms(P<0.05);肢体导联低电压和Ⅰ度房室传导阻滞分别为13例(41.9%)和7例(22.6%)。在31例患者中均记录到持续性室性心动过速(VT),其中15例(48.4%)为单形性,16例(51.6%)为多形性。经导管射频消融治疗者14例,即刻成功11例(78.6%),随访(18.3±10.2)个月,6例 VT 复发(54.5%);药物治疗17例,其中7例在置入心脏除颤器情况下用药,随访(35.6±19.0)个月,11例 VT 复发(64.7%),1例猝死。结论 ARVC 青、中年起病,胸前导联T波倒置、s波、V_(1～3)导联平均 QRS 时程≥110 ms 是其特征性心电图表现,经导管射频消融远期复发率高,药物预防远期效果不佳,心脏除颤器是值得选择的防治措施。
Objective To retrospectively analyze arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods According to the diagnostic criteria of the European Society of Cardiology in 1994, 31 patients with ARVC were selected and analyzed for clinical manifestations, electrocardiogram characteristics, treatment methods and follow-up efficacy. Results There were 27 males and 4 females in 31 cases. The first diagnosis was from 19 to 58 (34.7 ± 9.4) years old. 28 cases (90.3%) had palpitation, dizziness, 13 cases (41.9%) had history of syncope, 3 cases (9.7%) had syncope as the first symptom, and 1 case had sudden death from family history. Echocardiography and / or MRI, 29 cases showed right ventricular enlargement, including 2 cases of left ventricular enlargement. Resting electrocardiogram showed varying degrees of T wave inversion, mainly in the anterior thoracic aorta; 17 cases (54.8%) were found to have epsilon (ε) wave; 26 cases (83.9%) had an average QRS duration ≥110 ms, The QRS duration of QRS was (120.8 ± 13.7) ms and (99.4 ± 13.7) ms (P <0.05) in V_ (1 ~ 3) and V_ (4 ~ 5) Limb lead and I degree atrioventricular block were 13 (41.9%) and 7 (22.6%) respectively. Persistent ventricular tachycardia (VT) was recorded in 31 patients, of which 15 (48.4%) were monomorphic and 16 (51.6%) were pleomorphic. Among the 14 patients who underwent catheter ablation, 11 were immediate success (78.6%), followed up (18.3 ± 10.2) months, and 6 were VT recurrences (54.5%). Seventeen patients were treated with radiofrequency ablation, of which 7 were treated with defibrillation The patients were followed up (35.6 ± 19.0) months, 11 cases of VT recurrence (64.7%) and 1 case of sudden death. Conclusion The onset of ARVC in middle and young age, T wave inversion in thoracic aorta, s-wave, V 1 (1-3) lead QRS duration ≥110 ms is the characteristic electrocardiogram and the long-term recurrence rate after catheter ablation High, poor long-term drug prevention, defibrillator is worth the prevention and treatment measures.