致心律失常性右室心肌病ARVC

粉样变血色病心脏结节病致心律失常性右室心肌病?(ARVC)致心律失常性右室心肌病(ARVC)病变主要累及右室(RV),RV心肌不同程度地被脂肪或纤维脂肪组织代替,病变呈灶性或弥漫性,累及RV前壁漏斗部、心尖部及后下壁,临床主要:室性心律失常,猝死,右心衰竭致心律失常性右室心肌病(ARVC)病因不清先天性发育不良基因突变退性变引起心肌细胞变性坏死心肌慢性炎症,坏死为脂肪组织所代替致心律失常性右室心肌病(ARVC)超声心动图:(1)RV扩大,流出道增宽(2)RV运动异常或障碍,舒张期呈袋状膨突或呈室壁瘤样改变(3)RV调节束结构异常,肌小梁紊乱Figure1.EchocardiographicviewsfromprobandsmeetingTaskForceCriteriaforarrhythmogenicrightventricular(RV)dysplasia.(A)Rightventricularoutflowtract(RVOT)enlargementfromtheparasternallongaxisview,(B)RVOTenlargementfromtheparasternalshortaxisview,(C)apicalfour-chamberviewshowingafocalRVapicalaneurysm(arrows),(D)apicalfour-chamberviewshowingexcessivetrabeculations(arrows),and(E)apicalfour-chamberviewshowingahyperreflectivemoderatorband(arrow).AoVaorticvalve;LAleftatrium;LVleftventricle;RArightatrium.2005,45(6):860-865ARVC(ECHO)