致心律失常性右室发育不良的临床研究  

Clinical study on arrhythmogenic right ventricular dysplasia

作　　者：汪康平[1] 宋士良[1] 刘志华[1] 陈弹[1] 盛晓东[1] 叶云卿[1] 王惠芬[1] 蒋文平[1] 

机构地区：[1]苏州医学院附属第一医院心血管病研究室,215006

出　　处：《中华心律失常学杂志》1998年第1期10-13,共4页Chinese Journal of Cardiac Arrhythmias

摘　　要：目的研究致心律失常性右室发育不良(ARVD)患者的临床表现和预后。方法对1975年～1995年12例 ARVD 患者的临床和随访资料进行回顾性分析。结果室性心动过速(VT)发作时伴晕厥41.6%、休克50%、充血性心力衰竭(CHF)16.6%、心室颤动(VF)33.3%,VT 均为左束支阻滞(LBBB)图形。窦性心律时,右胸前导联 T 波倒置100%,心室激后波66.6%,心室晚电位(VLP)阳性100%,电生理研究(EPS)碎裂电位50%,诱发 VT83%。所有患者心脏均扩大,心胸比率平均0.60,右室内径(RVID)3.8 cm、右室流出道(RVOT)4.4 cm、伴左室腔扩大1例。平均随访7.2年,VT 发作期平均5.6年,缓解期平均9.3年,死亡率50%,猝死占66%。结论 ARVD 有家族群集倾向,持续性室性心动过速(SuVT)、猝死(SD)多见,CHF 少见,消除诱因和抗心律失常药物的应用,对防治恶性室性心律失常同样重要,本组平均病程可达13年,1例植入心律转复除颤器(ICD)已随访5年,至今仍存活。Objective To study the clinical manifestations and prognosis of patients with ayyhyth- mogenic right ventricular dysplasia(ARVD).Methods From 1975 to 1995,the clinical and follow-up data of 12 patients with ARVD were studied retrospectively.Results During ventricular tachyeardia(VT)at- tack,syncope was found in 41.6 %,shock in 50%,heart failure(CHF)in 16.6 %,ventricular fibrillation (VF)in 33.3%.The VTs were of LBBB configuration in all patients.During sinus rhythm,T wave inver- sion over right pericordial leads was shown in 100%,ventricular postexcitation waves were observed in 66.6% ,ventricular late potentials in 100% ,during EPS fractional potentials in 50% ,induced VT in 83%. All patients heart was enlarged,mean cardiothoracic ratio was 0.6,mean RV diastolic dimension 3.8 cm, RV outflow tract 4.4cm.One case was associated with LV enlargement.The average follow-up period was 7.2 years.10 patients with sustained VT(SuVT)had an average recurrent attack period of 5.6 years,6 pa- tients were known to have a relative remission period of 9.3 years.Total cardiac mortality was 50%,66% of which were sudden death(SD).Conclusion This disease usually has trends of family clusters.SuVT and SD are most common;CHF is less.Eliminating precipitating factors is as important as antiarrhythmic drugs for controlling malignant ventricular arrhythmias.In this group,mean clinical course was 13 years. One patient who had an ICD implanted has survived for more than 5 years.

关 键 词：VT ARVD 心律失常性右室发育不良 患者 CHF 随访 猝死 临床研究 心室晚电位 T波倒置 

分 类 号：R541[医药卫生—心血管疾病] R540[医药卫生—内科学]