兒科學(xué)-14先心病各論

心血管系統(tǒng)疾Cardiovascular韓波山東省立醫(yī)院兒二 性心臟病各房間隔缺（atrialseptaldefect,20%ofallCHD. 病理分型(1)第一孔型 孔)缺(ostiumprimumdefect占(2)第二孔型(繼發(fā)孔)缺（ostiumsecundum最常見類型,占所有ASD的(3)靜脈竇型缺損(sinusvenosus AtrialSeptalRedbloodcrossesdefecttoentertherightsideoftheheart-leadingtoincreasedflowtothe

AtrialSeptal TheMitral isalso andisNormalNormal&(alsocalledSeptalRedRedbloodcrossesdefecttoentertherightsideoftheheart-leadingtoincreasedflowtothe

AtrialSeptalDefectDeviceClosureSurgicalRepairNormalNormal&AtrialSeptalDefect–SinusVenosus3臨床（CLINICAL病史，哭鬧、 或心力衰竭，體格（Physical體型瘦收縮期雜音(systolicejectionmurmur)，肺 (splitandfixedS2)。 diastolicrumble)。心電圖①電軸右偏(Rightaxisdeviation②右室(Rightventricularhypertrophy,③不完全性右束支 pleterightbundlebranchblock,RBBB)V1導(dǎo)聯(lián)呈現(xiàn)rsR’圖X線檢查(X-ray心臟增大(cardiomegaly)(pulmonaryvascularmarkings(prominentmainpulmonaryartery超聲心動圖ASD的位置與大(sizeandlocationofthe(Indirectsignsofleft–to–rightshuntarepresent) 食道心導(dǎo)管檢查導(dǎo)管可通過ASD由右房進(jìn)入左房預(yù)后（NATURAL(1)<4歲，40%自然閉合（spontaneousclosure<3mm,100%,within33-8mm,80%,within18(2)心力衰竭（congestiveheartfailure(3)20s-30s發(fā)生肺動脈高(4)成人期發(fā)生房性心律紊亂(atrial(5)腦血管意外(cerebrovascular治療治療原不必限制體 非手術(shù)治療(Nonsurgicalclosure心臟介入手術(shù)（cardiacinterventional①繼發(fā)孔缺損②ASD肺靜脈的距離應(yīng)>5mm;④房間隔伸展徑大于

Deviceclosureofemiiemacrosa

septaldefectseptal經(jīng)導(dǎo)管關(guān)閉手術(shù)指征（1）（2）Highpulmonary手術(shù)時2~6yearsofCHFduringinfancydoesnotrespondtomedicalmanagement.率(mortality)腦血管意外（cerebrovascular 律失常（postoperativeAtrialSeptalDefect–Patch(ventricularseptaldefect,MostcommonCHD,25%ofall膜部或（membranous,perimembranous流出道（outlet/infundibular)肌小梁部（trabecularseptum:central marginalorapicalmusculardefect）5%-流入部（inletseptum）5%-VentricularSeptalSmallSmalldefectsproduceverylittledisturbancetoheartfunction

SeptalDefectModerateNormalheart&arteriesMuscularVSDNormalheart&arteriesDoublyCommittedRedbloodcrossesdefecttoentertherightsideoftheheart-leadingtoincreasedflowandpressureinthelungsNormalNormalheart&arteries

SeptalDefectSurgicalPAMuscularMultipleVSDsDoublyCommittedVSD

MidmuscularMidMuscularsurroundedby

MultipleVSDsDoublyCommittedVSDSmallVSD<0.5cm/m2ModerateVSD0.5～1cm/m2LargeVSD>1cm/m2動力型PH→梗阻型綜合(EisenmengerCLINICAL喂 (poorfeeding)呼吸急促、面色蒼白、生長發(fā)育延反復(fù)肺充血性心力衰竭Physical心前區(qū)隆L3,L4可觸及收縮期震顫（systolicthrillP2Agrade2-5/6regurgitantpansystolicmurmurisaudiableatthelowerleftAgrade1-3/6earlydiastolicmurmurofWithasmallVSD,theECGisWithamoderateVSD,左室肥厚WithalargeVSD,雙室肥厚（combinedventricularhypertrophy,CVH）X-rayCardiomegaly:LVH,PulmonaryvascularmarkingsareincreasedProminentmainpulmonaryarterysegmentThemember,sizeandlocationofthedefectEstimatePApressure;identifyotherassociateddefectsEstimatethemagnitudeoftheNATURAL（spontaneousclosure）Inletandinfundibulardefectsdoesnot.充血性心力衰竭（CHF）in梗阻性肺動脈高幾歲(teenageyears)時發(fā)生。（Infundibularstenosis→acyanotic endocarditis，IE)較少發(fā)CHFdigoxinand無PH時,無須限制體力活保持口腔衛(wèi)生,預(yù) 性心內(nèi)膜NonsurgicalAmplatzerVSDocclusion指征(indications):大于31.膜部和肌部2.VSD3.VSD距主動脈瓣的距離大于各種VSD封堵 指征1（QP/QS）2:1小型VSD在學(xué)齡前手證肺血管阻力與體循環(huán)阻力或有Eisenmengersyndrome

PA(forMultipleA‘Band’(madeoftape)isplacedaroundPulmonaryArterytorestrictthehighbloodflowandintheOtherOther‘SurgicalVentricularSeptalDefect動脈導(dǎo)管未（patentductusPDAoccursin15%ofall管窗AsmallDuctusproducesverylittledisturbancetoheartAsmallDuctusproducesverylittledisturbancetoheartCoilClosureLargePDANormalNormalheart&arteriesRedbloodcrosses‘Ductus’toenterthePulmonaryArtery-leadingtoincreasedflowandpressureinthelungs

PatentDuctus(PDA)-largeSmall小PDA無癥狀（ Physical差異 （differential心前區(qū)搏動增Asystolicthrillmaybepresentattheupperleftsternalborder水沖脈（BoundingperipheralAgrade1-4/6continuous(“machinery”)murmurattheuppersternalborder(L2)Apicaldiastolicrumble,resultingfromLAH,CVHorRVHwhenX-rayLAE,PulmonaryvascularmarkingsareincreasedProminentPAsegmentandhilarvesselswhenPHPDAsizeon肺動脈血氧含量高 心導(dǎo)管可通過PDAPatentDuctusArteriosusinanNATURAL大型PDA反復(fù) 和發(fā)展成肺動脈高壓可發(fā) 性心內(nèi)膜炎（IE)與動脈炎可發(fā)生動脈導(dǎo)管瘤樣（aneurysm）、鈣化及血栓形成消炎痛對足月兒PDA無無肺動脈高壓(pulmonaryarterial應(yīng)預(yù)防Nonsurgical>6PDA<2mmmultiplePDA>2mmAmplatzerPDAocclusiondevice各種CoilOcclusionoffromlegveinIVCtoRA,andthroughCoilOcclusionofCoilpassedintoDuctus,withCoilpassedintoDuctus,with2-3loopsinAorta&1-2inPAfibersincoilmakebloodform‘Clot’oncoilCoilOcclusionofAfter‘Coil’Ductusnowclosed SurgicalIndications.AnotomicexistenceofaPDAisanindicationforContraindications.Pulmonaryobstructivedisease肺動脈狹(pulmonarystenosis,7%ofallThree肺動脈瓣狹窄（valvularPS）most肺動脈瓣下狹窄（subvalvular肺動脈瓣上狹窄（supravalvularPS）is 肥臨床表現(xiàn)取決于肺動脈口的狹窄程度 流出道的發(fā)育情

ltyCriticalNormal&Normal&ofRight

Pulmonary (viewedfrom

Normal NormalNormalStenoticNormalStenotic

Pulmonary CLINICALInfantsandChildrenswithmildPSareusuallyasymptomaticExertionaldyspneaandeasyfatigabilitymaybepresentinpatientswithmodera severecases.HeartfailureorexertionalchestpainmaydevelopinseverecasesPhysical搏動胸骨左緣上方可觸及收縮期胸骨左緣上方可聞及收縮早期喀喇 ，P2音（systolicejectionmurmur）.新生兒重度PS可發(fā)生RightaxisdeviationRightventricularThedegreeofRVHontheECGcorrelateswiththeseverityofPSwithanrSR’patterninV1aretypicalX-rayNormalheartsizeinmildandmoderatecasesRVHinseverePulmonaryvascularmarkingsProminentmainPAThickpulmonaryvalvecuspswithrestrictedsystolicmotion(doming).Thesizeofthepulmonaryvalveannuluscanbeestimated.TheDopplerstudyforthepressuregradient(PG)acrossthestenoticvalve.10toNATURAL重度PS可發(fā)生偶爾發(fā)生IE(Infective重度PS可猝死（sudden除非重度PS應(yīng)預(yù)防Infectiveendocarditis經(jīng)皮球囊肺動脈瓣成形（percutaneousballoonvalvuloplasty，PBPV)適合于PG>30mmHg者。新生兒重度 ，應(yīng)緊急處理 BalloonValvuloplasty‘Balloon’CatheterfromlegveinthroughIVCtoRA,RVandBalloonBalloon‘Balloon’inflatedto‘Open’narrowvalve-AfterAfterValveValveafterAfterInterventionalcatheter單球囊雙球囊Indicationsand肺動脈瓣發(fā)育不良的漏斗部狹窄者（InfundibularPBPV不成功肺動脈瓣切開術(shù)（pulmonaryAleftGore-Texshunt(systemic-to–PA法 (tetralogyof10%ofallCHD,Themostcommoncyanoticheartdefectseeninchildren四種畸膜部VSD（perimembranous右室流出道狹窄（RVOT主動脈騎跨（aorta肥厚TetralogyofTightlyTightlyPulmonaryValandRV Aortic OverridesR>Lvia RVClicktoview

AbsentValveTetralogyof出生即可聞及心臟雜生后或不久即 （cyanosis勞力性氣促、蹲踞缺氧發(fā)作（hypoxicPhysicalCyanosis,tachypnea,andRVAsystolicthrillattheupperandmidleftsternalborders S2single;Along,loud(grade3-5/6)ejectiontypesystolicmurmuratthemidandupperleftsternalTheholosystolicregurgitantMurmur(L3、rightaxisX-rayCyanoticNormalheartDecreasedpulmonaryvascular右位主動脈弓（rightaorticAlarge,perimembranousVSDandoverridingoftheaortaRVOTDopplerstudiesestimatethepressuregradientacrossPA 左、和主動脈收縮壓基導(dǎo)管可由直接主動脈,表明動脈騎主動脈血氧飽和度明顯降TetralogyofFallotandRightAorticArchinan5143RightventricularoutflowtractobstructionOverridingseptalRightaortic

ANATURALInfantswithacyanoticTOFgradually缺氧發(fā)作（hypoxicspells/cyanotic腦膿腫（brain性心內(nèi)膜炎栓

醫(yī)師必須及 、治療缺氧發(fā)（hypoxic發(fā)作輕者，取胸膝臥位即可緩 0.05mg/Kg靜脈注射或心得安每次皮下注 0.1-0.2mg/Kg5%碳酸氫鈉1.5-5.0ml/Kg靜注常有缺氧發(fā)作者，心得安1-3mg/kg/d口服tidPBPV道狹窄（ 手術(shù)（palliativeshunt：體肺循環(huán)分流術(shù)BT根治6~12month術(shù)后并發(fā)癥

ClassicBlalock-TaussigRightsubclavianarterydetachedandsewntorightpulmonaryarteryOthertypesofThe‘ClassicBlalock-Taussig’shuntwasfirstperformedin