CAJ | 학술논문

目的探讨心室预激性扩张型心肌病的临床特征、诊断方法、发病机制及其预后.方法回顾性分析首都医科大学附属北京安贞医院小儿心脏中心2011年3月至2012年8月诊断的4例心室预激性扩张型心肌病的诊断过程,分析其临床、超声及电生理特点,随访其射频消融术后转归.结果 4例患儿体表心电图均提示为B型预激,成功消融靶点2例位丁右侧前间隔,另2例位丁右侧游离壁.M型超声示室间隔与左心室后壁呈不协调运动,二维超声检查发现室间隔基底段变薄且呈瘤样矛盾运动;斑点追踪技术发现左心室收缩不同步.经成功消融后,体格发育及体力活动量明显改善;超声提示左心室收缩协调,左心功能渐恢复止常,左心室舒张末径明显回缩至正常或接近正常.结论右侧前间隔或游离壁显性旁路可能对室壁运动及左心室功能产生不良影响,严重者可致扩张型心肌病.心室预激致左心室收缩不同步可能是其最重要的发病机制.心室预激性扩张型心肌病为射频消融的适应证,预后良好.
목적 탐토심실예격성확장형심기병적림상특정、진단방법、발병궤제급기예후.방법 회고성분석수도의과대학부속북경안정의원소인심장중심2011년3월지2012년8월진단적4례심실예격성확장형심기병적진단과정,분석기림상、초성급전생리특점,수방기사빈소융술후전귀.결과 4례환인체표심전도균제시위B형예격,성공소융파점2례위정우측전간격,령2례위정우측유리벽.M형초성시실간격여좌심실후벽정불협조운동,이유초성검사발현실간격기저단변박차정류양모순운동;반점추종기술발현좌심실수축불동보.경성공소융후,체격발육급체력활동량명현개선;초성제시좌심실수축협조,좌심공능점회복지상,좌심실서장말경명현회축지정상혹접근정상.결론 우측전간격혹유리벽현성방로가능대실벽운동급좌심실공능산생불량영향,엄중자가치확장형심기병.심실예격치좌심실수축불동보가능시기최중요적발병궤제.심실예격성확장형심기병위사빈소융적괄응증,예후량호.
Objective To discuss the characteristics,diagnosis,mechanism and the prognosis of dilated cardiomyopathy (DCM) induced by accessory pathway (AP).Methods The clinical,electrophysiological and echocardiographic characteristics in four cases,who were diagnosed as AP-induced DCM in the Department of Pediatric Cardiology of Beijing Anzhen Hospital,Capital Medical University from Mar.2011 to Aug.2012,were analyzed before and after ablation.Results The electrocardiograms of the 4 patients all indicated type B ventricular preexcitation.The locations of the APs were the right-sided anteroseptum and the free wall.Dyschronous contractions between posterior wall of left ventricle and interventricular septum were demonstrated by M-Mode echo.The basal segments of the interventricular septum turned thin and moved similar to an aneurysm,with typical bulging during end-systole,which was observed in all cases by two-dimension echo.Dyschronous left ventricular contraction was shown by speckle tracing technique.All patients received successful radio frequency current ablations.Their physical activities and growth improved greatly in the 4 cases.The echocardiographic data demonstrated that their left ventricular contraction recovered to synchrony shortly after the ablation,left ventricular ejection fraction recovered to normal and left ventricular end diastolic diameter decreased to almost normal gradually during the follow-up.Conclusions Overt right-sided APs localized in anteroseptum or free wall may have adverse effects on ventricular wall motion and left ventricular function.They can even result in DCM.Dyssynchronous ventricular contraction induced by right-sided overt accessory pathway may be the vital mechanism.AP-induced DCM is an indication for ablation with good prognosis.