先天性心脏病y1

CongenitalHeartDisease(CHD)ShengjingHospitalPediatricsYuXuexinIntroductionCHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater.

Incidence:6.9‰inaliveneonatal.150,000neonatalsufferfromCHDinChinaperyear.Newtreatments：catheterization、developmentofoperation,etc.

ObjectandRequestFamiliarwiththeetiologyandclassificationofCHD.Masterthehemodynamics、clinicalmanifestationanddiagnosisofcommoncomplicationsinVSD,ASD,PDAandTOF.EtiologyInternalfactors：genemutationorchromosomeaberration.Adefectinthelongarmofchromosome22associatedwiththeDiGeorge,Shprintzen,andconotruncalanomalyfacesyndromes.ThesechildrentendtohaveeitherinterruptedaorticarchorconotruncalabnomalitiessuchasTOFordoubleoutletrightventricle.Externalfactors:intraureteralinfection(rubellavirus)、ray、drug、metabolicdiseases、intraureteralhypoxia.Theincidenceinchildrenofaffectedmothersmaybeashighas10%-15%.Classificationleft-to-rightshunts

CyanosismaybeVSD、PDA、ASD

right-to-leftshunts

non-shuntsCyanosis

TOF、dislocationofmainarteryPulmonaryarterystenosis、aorticstenosisBasedonshuntbetweenrightandleftheartPatentductusarteriosus

PDACommonCHDinClinicAtrialseptaldefect

ASDVentricularseptaldefect

VSDTetralogyofFallot

TOF1234VentricularSeptalDefect（VSD）1、membranedefect85%2、musculardefect3、funneldefect10%20-50％VSDcancloseupwithouttreatment.1、minordefect2、mediadefect3、majordefectAnatomyMostcommon,30%inCHD.PathobiologyRV

blood↑,pulmonaryhypertension,persistentcyanosis(Eisenmengersyndrome)LVblood↓,bodycirculation↓HemodynamicsBeforepulmonaryhypertension

RARV（blood↑）Pulmonaryartery(dilation)Pulmonarycirculation

(congestion)RV(dilation)LA(hypertrophy)LV(hypertrophy)（volumeejection↓）bodycirculationBloodvolume↓shuntHemodynamicsBodycirculation（mixedblood）RALAPulmonaryArterydilationRV（Dilation)AfterpulmonaryhypertensionLVDynamicPulmonaryhypertesionObstructivepulmonaryhypertesionshuntClinicalManifestationSymptoms：frequentrespiratoryinfections,growslowly,poorweight,dyspnea,exerciseintolerance,fatigue,hoarseness(PApressrecurrentlaryngealnerve).Signs：pansystolicmurmur,loudⅢ-Ⅳ,harshwideconduction,3rd-4thintercostalspaceatleftsternalborder.P2accent.ExaminationX-ray:aorticknobsmallerthannormalmainPAsegmentprotrusionLVenlargementincreaselungmarkingsECG:LVhypertrophyUS:interventricularseptumintermittedComplicationsandTreatmentComplicationsbronchopneumoniacongestiveheartfailurepulmonaryedemainfectiousendocarditisTreatmentminordefect：mayavoidoperation.mediadefect：operationat5-6yearsold.majordefectwithcomplications:operationat6m-2y.

AtrialSeptalDefect（ASD）5%-10%Bioanatomy：1.ostiumprimum：15%.2.ostiumsecundum：mostcommon，75%.3.venoussinus：5%.4.coronaryvenoussinus：2%.AtrialSeptalDefect（ASD）HemodynamicVenaecavaeRA(dilation)RV(dilation)Pulmonary

circulation

congestionArteriole

spasm

、thickenedRighttoleftshuntEisenmengersyndromePulmonary

veinLALV(blood)↓BodycirculationischemiaASDEtibiologyPulmonarycirculationbloodvolumeincreaseBodycirculationbloodvolumedecreaseClinicalManifestationSymptoms：similartoVSD.Auscultation：S1accent，P2accentejectivesystolicmurmurat2ndleftintercostalspace,loudⅡ-Ⅲ(pulmonaryarteryvalverelativelynarrow).TestX-ray：RAandRVenlargement.“hilusdance”,pear-shapedheart.ECG：rightaxisdeviation，incompleterightbundlebranchblock.B-US：RAandRVenlargement,paradoxicalmovementofinterventricularseptum.ComplicationsandTreatmentComplicationsbronchopneumonia、heartfailure.Treatmentoperationbeforeschoolcatheterization15%ofCHDPatentDuctusArteriosus（PDA）AnatomyTubulartypeInfundibulartypeWindowtypePathobiology1、Pulmonarycirculationcongestion2、Bodycirculationischemia3、Whenpulmonarypressurehigherthanaorta,righttoleftshuntoccurs,lowerlimbscyanosis—differentialcyanosisPDAClinical

manifestationSyndromes：similartoASDandVSDSigns：continuousmachinerymurmuratthe2ndleftintercostalspace,occupiesthewh