小儿心脏疾病

CardiovascularDiseasesinChildren

（GeneralIntroduction

）（黄国英）HUANGGuo-ying,M.D.Professor,DepartmentofPediatricsFudanUniversityShanghaiMedicalCollegegyhuang@教学要求1.熟悉心血管胚胎发育、先心病病因及病理机制2.了解胎儿循环特点及出生后变化3.熟悉小儿循环系统常用检查方法4.掌握先天性心脏病的分类5.了解小儿先心病治疗方法复习与思考题1.先心病的分类及其代表性疾病2.先心病的临床表现及常用的检查方法

3.心脏胚胎发育与先心病的关系Cardiovascular

DiseasesinChildrenCongenitalheartdiseases

Viralmyocarditis Rheumaticheartdisease Cardiomyopathy KawasakiDisease Arrhythmia Congestiveheartfailure Pericarditis Infectiveendocarditis Etc….FetalHeartDevelopmentFormationandLoopingofPrimitiveHeartTube胚胎第3周胚胎第4周胚胎第5-6周胚胎第7-8周FormationofEndocardialCushionAVcanalposteriorrightAVorificeanteriorleftAVorifice胚胎第3周胚胎第4周胚胎第5-6周胚胎第7-8周FormationofEndocardialCushionSeptationofAtriaandVentriclesostiumprimum1＝LA胚胎第3周胚胎第4周胚胎第5-6周胚胎第7-8周2＝LV3＝RA4＝RV5＝septumprimum6＝septumsecundum7＝endocardialcushionostiumsecundumforamenovale8,9＝ventricularseptaInterventricularforamenSeptationofVentriclesIVSiscomposedofthreeparts：①muscularseptum②cushioncomponent③A-Pseptum componentSeptationofGreatArteriesAOPAPAAOPAPAAOAOPAPAPAPAMalpositionofGreatArteriesTOFTGADORVTaussig-BingEvolvementofPharyngealArchesobsoletesoonafterbirthcarotidarteriessubclavianA.brachiocephalicA.ductusarteriosusbranchesofpulmonaryartery1stpair2ndpair3rdpair4thpair5thpair6thpairFetalCirculation

&ChangesafterBirthafterbirthbeforebirthCongenitalHeartDiseasesDefinition：

Cardiovascularmalformationspresentduringembryonicstages

（胚胎期发生的心血管畸形）Prevalence： －7~8/1000newborns（reporteddata） －6.87/1000newbornsinShanghai（Epidemiologicsurveyduring1989～1991）EtiologicConsiderationsHereditaryfactors(Associatedwith15%CHD)Trisomiesofchromosome21,18,15,13Deletionofchromosome22q11

（22号染色体长臂q11区域微小缺失）Genedefects：－DefectsofElastininWilliam’ssyndrome－MutationsofFibrillininMarfan’ssyndrome－MutationsofTbx5inHolt-Oramsyndrome－MutationsofCx43inhypoplasticleftheartEtiologicConsiderationsEnvironmentalfactorsExposedtothefollowingfactorsduringthefirsttrimesterofpregnancy－Viralinfections:rubella,influenza,enterovirus,parotitis－Physicalandchemicalfactors:drugs,radiation,alcohol,tobacco－Maternitydiseases:diabetes,connectivetissueproblems,hypertensionsyndromePathogenesisClassificationofCHD

BasedonHemodynamicsLeft-to-rightshuntlesions： －VSD,ASD,PDARight-to-leftshuntlesions： －TOF,D-TGA,TANon-shuntlesions： －PS,AS,CoALeft-to-rightLesionsPatentDuctusArteriosusAtrialSeptalDefectVentricularSeptalDefectRight-to-leftshuntlesionsTetralogyofFallotTranspositionoftheGreatArteriesTricuspidAtresiaNon-shuntlesionsPulmonaryStenosisAorticStenosisCoarctationoftheaortaRequiredfromThisLessonTounderstandtheembryologicaldevelopmentofheart,causesofCHDandthepathogenesisToknowthecharacteristicsoffetalcirculation&changesafterbirthTounderstandthediagnosticmethodsforCHDTounderstandfullytheclassificationofCHDToknowtheprincipleoftreatmentofCHDDiagnosticToolsforCHDHistoryTakingFrommotherandfamily:

－Historyofmotherduringpregnancy

－Abnormalhistoryofpreviouspregnancy

－FamilyhistoryofCHD,chromosomediseasesFromthechild

－Recurrentpneumonia

－Cyanosis

－Heartmurmurfoundbefore

－Cardiacdysfunction：feedingdifficulty,tachypnea, sweating,edema

－Others：skinny,hoarsenesswhencrying CardiacExaminationCardiacExaminationAuscultation：－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,apexphase SM,DM,CM SM,shortquality harsh softintensity >gradeII <gradeIIIradiation extensive localizedvariation

less

moreNon-cardiacExaminationArterialpulse:

－rate,rhythm,quality,amplitude, uniformityatlimbsArterialbloodpressure:Extremities： －clubbingoffingers&toes,edemaAbdomen:

－hepatomegaly,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-dimensionalEchocardiographyDopplerEchochardiographyThree-dimensionalEchocardiographyTransesophagealEchocardiographyFetalEchocardiographyM-modeEchocardiogramTwo-dimensionalEchocardiogramDopplerEchochardiogramPulsedDopplerEchochardiographyContinuous-waveDopplerEchochardiographyColorDopplerEchochardiographyPulsedDopplerEchochardiogramContinuous-waveDopplerEchochardiogramPG＝4xVmax2

ColorDopplerEchochardiogramReal-timeThree-dimensionalEchocardiogram

MagneticResonanceImagingValuabletoolintheevaluationofCHDParticularlyintheimagingofvascularstructuresofthethoraxSpiralComputedTomographyAnothervalu