兒童先天性心臟病CardiovascularDiseasesinChildren英文

CongenitalHeartDiseasesinChildren

（GeneralIntroduction）Cardiovascular

DiseasesinChildrenCongenitalheartdiseases

Viralmyocarditis Rheumaticheartdisease Cardiomyopathy KawasakiDisease Arrhythmia Congestiveheartfailure Pericarditis Infectiveendocarditis Etc….CongenitalHeartDiseasesDefinition：

Cardiovascularmalformationspresentduringembryonicstages

CHDaccountsfornearly1/3ofallmajorcongenitalanomalies.25%arelifethreateningandrequireearlyintervention.

TheprevalenceofCHDininfancyisestimatedat6-8per1000livebirths.(Indian)

－

7~8/1000newborns（reporteddata）

(China)

－

6.87/1000newbornsinShanghai（Epidemiologicsurveyduring1989～1991）

PrevalenceEtiologicConsiderationsHereditaryfactors(Associatedwith15%CHD)Trisomiesofchromosome21,18,15,13Deletionofchromosome22q11

Genedefects：－DefectsofElastininWilliam’ssyndrome－MutationsofFibrillininMarfan’ssyndrome－MutationsofTbx5inHolt-Oramsyndrome－MutationsofCx43inhypoplasticleftheartEtiologicConsiderationsEnvironmentalfactorsExposedtothefollowingfactorsduringthefirsttrimesterofpregnancy－Viralinfections:rubella,influenza,enterovirus,parotitis

－Physicalandchemicalfactors:drugs,radiation,alcohol,tobacco－Maternitydiseases:diabetes,connectivetissueproblems,hypertensionsyndromePathogenesisHypostasis：－Heartdevelopmentisaverycomplexprocessinvolvingmanycoordinatedsteps－AbnormalembryologicaldevelopmentoftheheartleadstoCHDMechanism：－GeneticbasisinteractedwiththeenvironmentalfactorsplayrolepossiblyinmostcasesFetalHeartDevelopmentFormationofprimitivehearttubeLoopingofprimitivehearttubeFormationofendocardialcushionFormationofinteratrialseptumFormationofinterventricularseptumSeptationofaorticandpulmonaryarteries

FormationandLoopingofPrimitiveHeartTube3weeksofintrauterinelife4weeksofintrauterinelife5-6weeksofintrauterinelife7-8weeksofintrauterinelife11

FetalCirculation

&ChangesafterBirthafterbirthbeforebirthDifferencesbetweenthefetalandpostnatalcirculationPresenceofplacentalcirculation,whichprovidesgasexchangeforthefetus.AbsenceofgasflowofbloodtothelungsandthuslittlepulmonaryvenousreturntoleftatriumPresenceofductusvenosus,joiningtheportalveinwiththeinferiorvenacava,providingalowresistancebypassforumbilicalvenousbloodtoreachtheinferiorvenacavaWidelyopenforamenovaletoenableoxygenatedblood(throughumbilicalveins)toreachtheleftatriumandventriclefordistributiontothecoronariesandthebrainWideopenductusarteriosustoallowrightventricularbloodtoreachthedescendingaorta,sincelungsarenon-functioning.Circulatoryadjustmentsatbirth-transitionalcirculationLossofplacentalcirculationandclampingoftheumbilicalcord,afterbirth,resultsinasuddenincreaseinsystemicvascularresistancewiththeexclusionofthelowresistanceplacentalcirculation.ClassificationofCHD

BasedonHemodynamicsLeft-to-rightshuntlesions：

－VSD,ASD,PDARight-to-leftshuntlesions：

－TOF,D-TGA,TANon-shuntlesions：

－PS,AS,CoALeft-to-rightLesionsPatentDuctusArteriosusAtrialSeptalDefectVentricularSeptalDefectRight-to-leftshuntlesionsTetralogyofFallot

TranspositionoftheGreatArteriesTricuspidAtresiaNon-shuntlesionsPulmonaryStenosisAorticStenosisCoarctationoftheaortaClinicfeaturesCyanosisDifficultfeedingandpoorgrowthDifficultbreathingFrequentrespiratoryinfectionsSpecificsyndromesClinicfeatures---CyanosisSeverecaseCryorexertsfeedingClinicfeatures--DifficultfeedingandpoorgrowthParentscomplainthatthechildhasdifficultywithfeeds.Slowfeeding,smallvolumesconsumedGrowthrateisnotappropriateforage.(growthretardationaffectsweightmorethanheight.)Clinicfeatures--DifficultbreathingTachypneaRespiratoryrates

>60/min<2mon>50/min>2mon

>40/min>12monClinicfeatures--FrequentrespiratoryinfectionsFrequentSevereLongdurationDifficulttotreatClinicfeatures--SpecificsyndromesTrisomy21Table15-7P401DiagnosticToolsforCHD

－Historytaking

－Physicalexamination

－Electrocardiography（心電圖檢查）

－ChestRoentgenography（X線胸片）

－Echocardiography（超聲心動(dòng)圖）

－Magneticresonanceimaging（磁共振成像）

－ComputedTomography(計(jì)算機(jī)斷層攝影術(shù))

－Cardiaccatheterization（心導(dǎo)管檢查）

－Angiocardiography（心血管造影）HistoryTakingFrommotherandfamily:

－Historyofmotherduringpregnancy

－Abnormalhistoryofpreviouspregnancy

－FamilyhistoryofCHD,chromosomediseasesFromthechild

－Recurrentpneumonia

－Cyanosis

－Heartmurmurfoundbefore

－Cardiacdysfunction：feedingdifficulty,tachypnea, sweating,edema

－Others：

skinny,hoarsenesswhencrying CardiacExaminationInspection：

－Prominenceofprecordium（心前區(qū)隆起）

－Increasedcardiacactivity（心臟搏動(dòng)彌散）

－Jugularvenousdistension（頸靜脈怒張）Palpation：

－Apeximpulse(心尖搏動(dòng)）

－Heaveofimpulse（抬舉樣搏動(dòng)）

－Precordialthrill（震顫）Percussion：

－EstimationofsizeandlocationoftheheartCardiacExaminationAuscultation：－Rateandrhythmofheartbeats－Normalheartsounds: S1,S2(A2&P2),S3,S4－Abnormalheartsounds: splitting,intensity,ejectionclicks,S3,S4－Murmurs: systolic,diastolic,continuous－PericardialfrictionrubCardiacExaminationHeartRateandRhythmNeonate 120～140bpmInfancy 110～130bpmToddler’sage 100～120bpmPreschoolage 80～100bpmSchoolage 70～

90bpmCardiacExaminationDifferentialdiagnosisofheartmurmur CHD Innocentlocation precordium 2～4LSB,apex

phase SM,DM,CM SM,short

quality harsh soft

intensity >gradeII <gradeIII

radiation extensive localized

variation less moreNon-cardiacExaminationArterialpulse:

－rate,rhythm,quality,amplitude, uniformityatlimbsArterialbloodpressure:Extremities：

－clubbingoffingers&toes,edemaAbdomen:

－h(huán)epatomegaly,spleenomegalyArterialBloodPressureUpperlimbs：

－SystolicBP(mmHg)=age×２+80

－DiastolicBP(mmHg)=2/3×BPsLowerlimbs：

－BPis20mmHghigherAbnormalBP：

－20mmHghigherorlowerthannormalElectrocardiogramEKGisessentialinassessinghypertrophyofatriaorventricles,arrhythmiasanddisordersofconductivesystemEnlargementofAtriaEnlargementofleftatrium：－P-wave≥0.09secinduration－DoublepeaksofP-wavewithintervalof0.04secormoreEnlargementofrightatrium：－PII≥0.25mvinamplitude－PV1

≥0.20mvinamplitudeEnlargementofLeftVentricleRv5≥3.0mvinamplitudeSv1≥1.5mvinamplitudeRv5+Sv1≥4.5mvinamplitudeQRScomplexinleadV1shownasQSEnlargementofRightVentricleThefollowingsignsshowninleadV1:

－

QRScomplexpresentsasqR

－R>0.7mv

－rsR’complexwithR’>1.5mv

－RscomplexwithR/sratioasfollows：<1yr >51-3yr >2.53-5yr >25-12yr >1.5>12y >1RoentgenogramChestX-rayisessentialintheevaluationofheartsizeandpulmonaryvascularityEchocardiographyThemajornoninvasivediagnosticmethodforCHDTodefineanatomy,function,chamber&vesselsize,andvalveabnormalitiesModelitiesofEchocardiography

M-modeEchocardiographyTwo-dimensionalEchocardiographyDopplerEchochardiography

Three-dimensionalEchocardiographyTransesophagealEchocardiographyFetalEchocardiographyM-modeEchocardiogramTwo-dimensionalEchocardiogramDopplerEchochardiogramPulsedDopplerEchochardiography

Continuous-waveDopplerEchochardiography

ColorDopplerEchochardiography

PulsedDopplerEchochardiogram

ColorDopplerEchochardiogramReal-timeThree-dimensionalEchocardiogram

MagneticResonanceImagingValuabletoolintheevaluationofCHDParticularlyintheimagingofvascularstructuresofthethoraxSpiralComputedTomographyAnothervaluabletoolintheevaluationofvascularstructuresofthethoraxCardiacCatheterizationPerformedfortheneedoffurtheranatomicorphysiologicinformationPerformedforthepurposeoftreatmentinthecatheterizationlaboratoryCardioangiography

Nadas’Criteria—OnemajorortwominorcreteriaMajorcreteriaSystolicmurmurgrateIIIormoreinintensityDiastolicmurmurCentralcyanosisCongestivecardiacfailureMinorcreteriaSystolic