先天性心臟病病理生理非紫紺型先天性心臟病動(dòng)脈導(dǎo)管未閉10%房間隔缺損10%室間隔缺損20%房室間隔缺損2-5%主動(dòng)脈-肺動(dòng)脈間隔缺損（少見）主動(dòng)脈竇瘤破裂冠狀動(dòng)脈起源于肺動(dòng)脈冠狀動(dòng)脈漏肺動(dòng)脈狹窄主動(dòng)脈弓中斷1%主動(dòng)脈縮窄10%晚發(fā)紫紺肺動(dòng)脈血流增加肺動(dòng)脈高壓，血管變性2021/4/272紫紺型先天性心臟病Fallot四聯(lián)癥10%合并其他畸形的右室流出道狹窄肺動(dòng)脈狹窄10%肺動(dòng)脈瓣閉鎖5%右室雙出口完全性肺靜脈畸形引流單心房、單心室三尖瓣閉鎖3%EBSTEIN畸形0.5%大動(dòng)脈轉(zhuǎn)位5%-8%共同動(dòng)脈干3%左心發(fā)育不良綜合征2%2021/4/273先天性心臟病診斷病史和體征（喂養(yǎng)困難、多汗、呼吸困難、紫紺、蹲踞等）體格檢查（杵狀指、脈搏不一致、心臟擴(kuò)大、心前區(qū)隆起或震顫、異常心音、收縮期雜音、肝脾腫大）X-線（心影擴(kuò)大、肺血增多、肺血正常、肺血減少、內(nèi)臟異位）心電圖超聲心動(dòng)圖經(jīng)食道超聲心動(dòng)圖三維超聲、MRI、CT、多排CT、數(shù)字血管減影心導(dǎo)管2021/4/274先天性心臟病的術(shù)前評(píng)估主要危險(xiǎn)因素心臟及大血管的畸形程度——決定手術(shù)的難度。常見危及生命的畸形是肺動(dòng)脈發(fā)育不良和心室腔過小，傳導(dǎo)束和冠狀動(dòng)脈的畸形增加手術(shù)的難度。肺動(dòng)脈高壓（原發(fā)或繼發(fā)）的病理改變——決定術(shù)后肺循環(huán)功能和回心血量。正常肺血管阻力為2.5-3.75woods,收縮壓小于28mmHg，當(dāng)收縮壓大于70mmHg，吸氧后全肺阻力不減少，動(dòng)脈血氧飽和度小于85%，全肺血管阻力大于18woods，病人失去手術(shù)機(jī)會(huì)

2021/4/2752021/4/276svcIVCRARVPALALVAoPV2021/4/2772021/4/278TAPVC2021/4/2792021/4/27102021/4/2711PDA2021/4/27122.肺動(dòng)脈環(huán)縮術(shù)2021/4/2713LocationsofVSDinDORVSubaorticSubpulmonaryDoublycommittedNon-committed

10.9%(16/146)

2021/4/27142021/4/27152021/4/2716AORTICOPULMONARY-WINDOW2021/4/2717VictorEisenmenger,18641932,GermanphysicianIrreversibleseverepulmonaryarterialhypertensionresultingfromexcessivepulmonarybloodflowoveralongperiod.Thistermisnowusedtodescribetheclinicalsyndromeandpathophysiologyresultingfromreversalofanylefttorightshunt.Itcanbeproducedbyatrialseptaldefect,ventricularseptaldefect,patentductusarteriosus,atrioventricularseptaldefect(atrioventricularcanal),truncusarteriosus,aorticopulmonarywindowandotherlesions.2021/4/27182021/4/27192021/4/27202021/4/27212021/4/2722coarctationofAorta2021/4/27232021/4/2724svcRARVIVCPAAoLALVPV2021/4/2725Perioperativecollateralembolization!

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