致心律失常性右室心肌病20例临床分析

A clinical study of arrhythmogenic right ventricular cardiomyopathy

目的:探讨致心律失常性右室心肌病(ARVC)的临床和心电图特点。方法:收集、查阅2002-2005年20例诊断为ARVC患者入院病例资料,常规行临床资料、心电图、超声心动图、X线胸片、24h动态心电图及心内电生理检查的资料分析。结果:20例ARVC患者中有晕厥发作(65%)、心悸(100%)、频发室性早搏(100%)、右心室源性短阵室速(85%)。20例患者常规心电图检查中15例(75%)有右束支传导阻滞(RBBB),12例(60%)电轴右偏,7例(35%)出现Epsilon波,17例(85%)胸前导联V1～V3T波倒置,13例(65%)V1导联QRS波时限≥110ms,12例(60%)(V1+V2+V3导联QRS波时限之和)/(V4+V5+V6导联QRS波时限之和)比值>1.2。在无RBBB时,18例(90%)胸前导联V1～V3均可见延长的S波升支≥55ms。超声心动图示右心房内径为(48.00±8.81)mm,右心室内径为(50.90±10.30)mm,右心功能减退,射血分数为0.301±0.090。结论:ARVC多有发作性晕厥,心电图上可出现频发室早及呈左束支传导阻滞型室速,窦性心律时伴RBBB型或电轴右偏,Epsilon波和右胸导联QRS间期延长,长时间V1～V3T波倒置且无动态变化。超声心动图可发现右心室、右心房增大,右室与左室舒张末期内径之比增大,室壁变薄,右室肌小梁消失或紊乱,右室功能减退。

Objective To explore the clinical and electrocardiographic features in patients with arrhythmogenic right ventricular cardiomyopathy （ARVC）. Methods The clinical data, ECG, routine echocardiogram （UCG）, chest radiographs, Hoher recordings, and intracardiac eiectrophysiological studies were reviewed in 20 ARVC patients who had been hospitalized during 2002 to 2005. Results All the patients experienced palpitation attack and frequent ventricular premature beats （VPBs）, 13 of them （65%） had history of syncope, and 17 （85%） had ventricular tachycardia （VT） of right ventricular origin. 15 of the patients （75%） had right ventrieular bundle branch block （RBBB） on ECG, 7 （35%） developed epsilon waves, T-wave inversions occurred in 17 patients （85%） in the precordial leads V1 to V3, and QRS duration≥ 110 ms in V1 was present in 13 patients （65%）. The ratio of the sum of QRS durations in V1 - V3 to that in V4-V6 was greater than 1.2 in 12 patients （60%）. A prolonged S-wave upstroke≥ 55 ms in V1 to V3 was found in 18 patients （90%） in the absence of RBBB. UCG showed that the right atrium was （48.00± 8.81） mm in diameter and the right ventricle was （50.90 ± 10.30） ram. The right ventricular function was decreased with an ejection fraction of 0.301 ±0.090. Conclusions ARVC can be associated with episodic syncope. ECG shows frequent VPBs, VT with a LBBB pattern, sinus rhythm with RBBB or right axis deviation, epsilon waves, prolonged QRS complexes in the right precordial leads, and delayed, unchanged T-wave inversions in V1-V2; UCG reveals enlarged right atrium and ventricle, an increased ratio of right to left ventricular end-diastolic diameter, thinner ventricular wall, absence or disarray of trabeculae carneae, and a decline in the right ventricular function.