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,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,2021/3/12,*,武汉亚洲心脏病医院,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,2021/3/12,*,先天性心脏病病理生理,2021/3/12,1,先天性心脏病病理生理2021/3/121,非紫绀型先天性心脏病,动脉导管未闭,10%,房间隔缺损,10%,室间隔缺损,20%,房室间隔缺损,2-5%,主动脉,-,肺动脉间隔缺损 （少见）,主动脉窦瘤破裂,冠状动脉起源于肺动脉,冠状动脉漏,肺动脉狭窄,主动脉弓中断,1%,主动脉缩窄,10%,晚发紫绀,肺动脉血流增加,肺动脉高压，血管变性,2021/3/12,2,非紫绀型先天性心脏病动脉导管未闭 10%晚发紫绀肺动脉血,紫绀型先天性心脏病,Fallot,四联症,10%,合并其他畸形的右室流出道狭窄,肺动脉狭窄,10%,肺动脉瓣闭锁,5%,右室双出口,完全性肺静脉畸形引流,单心房、单心室,三尖瓣闭锁,3%,EBSTEIN,畸形,0.5%,大动脉转位,5%-8%,共同动脉干,3%,左心发育不良综合征,2%,2021/3/12,3,紫绀型先天性心脏病Fallot四联症,先天性心脏病诊断,病史和体征 （喂养困难、多汗、呼吸困难、紫绀、蹲踞等）,体格检查 （杵状指、脉搏不一致、心脏扩大、心前区隆起或震颤、异常心音、收缩期杂音、肝脾肿大）,X-,线（心影扩大、肺血增多、肺血正常、肺血减少、内脏异位）,心电图,超声心动图,经食道超声心动图,三维超声、,MRI,、,CT,、多排,CT,、数字血管减影,心导管,2021/3/12,4,先天性心脏病诊断病史和体征 （喂养困难、多汗、呼吸困难、紫,先天性心脏病的术前评估,主要危险因素,心脏,与,大血管的畸形程度,决定手术的难度。,常见危及生命的畸形是肺动脉发育不良和心室腔过小，传导束和冠状动脉的畸形增加手术的难度。,肺动脉高压（原发或继发）的病理改变,决定术后肺循环功能和回心血量。,正常肺血管阻力为,2.5-3.75woods,收缩压小于,28mmHg,，当收缩压大于,70mmHg,，吸氧后全肺阻力不减少，动脉血氧饱和度小于,85%,，全肺血管阻力大于,18woods,，病人失去手术机会,2021/3/12,5,先天性心脏病的术前评估主要危险因素 2021/3/125,2021/3/12,6,2021/3/126,svc,IVC,RA,RV,PA,LA,LV,Ao,PV,2021/3/12,7,svcIVCRARVPALALVAoPV2021/3/127,2021/3/12,8,2021/3/128,TAPVC,2021/3/12,9,TAPVC2021/3/129,2021/3/12,10,2021/3/1210,2021/3/12,11,2021/3/1211,PDA,2021/3/12,12,2.,肺动脉环缩术,2021/3/12,13,2.肺动脉环缩术2021/3/1213,Locations of VSD in DORV,Subaortic,Subpulmonary,Doubly committed,Non-committed,10.9%(16/146),2021/3/12,14,Locations of VSD in DORVSubaor,2021/3/12,15,2021/3/1215,2021/3/12,16,2021/3/1216,AORTICOPULMONARY-WINDOW,2021/3/12,17,AORTICOPULMONARY-WINDOW2021/3/,Victor Eisenmenger,18641932,German physician,Irreversible severe,pulmonary arterial hypertension,resulting from excessive pulmonary blood flow over a long period.This term is now used to describe the clinical syndrome and pathophysiology resulting from reversal of any left to right shunt.It can be produced by,atrial septal defect,ventricular septal defect,patent ductus arteriosus,atrioventricular septal defect,(atrioventricular canal),truncus arteriosus,aorticopulmonary window,and other lesions.,2021/3/12,18,Victor Eisenmenger,18641932,2021/3/12,19,2021/3/1219,2021/3/12,20,2021/3/1220,2021/3/12,21,2021/3/1221,2021/3/12,22,2021/3/1222,coarctation of Aorta,2021/3/12,23,coarctation of Aorta 2021/3/12,2021/3/12,24,2021/3/1224,svc,RA,RV,IVC,PA,Ao,LA,LV,PV,2021/3/12,25,svcRARVIVCPAAoLALVPV2021/3/122,Perioperative collateral embolization!,simplify the operative complexity,reduce the operative trauma,2021/3/12,26,Perioperative collateral embol,2021/3/12,27,2021/3/1227,Hypoplastic Left Heart Syndrome,2021/3/12,28,Hypoplastic Left Heart Syndrom,
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