小儿心脏病英语

PediatricandCongenitalHeartDisease

GeneralintroductionsKunSunM.D.XinhuaHospitalHeartStructureHeartisancomplexspatialstructureDifferencesinthepathophysiologyofcardiacdiseasePediatrics:congenitalheartdiseaseAdults:atheroscleroticheartdisease

AwordaboutdiagnosisClinicalpresentation--raisingthesuspicionImageisEVERYTHING Mainstay:echocardiographyechoanatomyandhemodynamicsCardiaccatheterizationimaginghemodynamicsinterventionStagesofHeartFormation1.Earlybloodvesselformation1)Intraembryonicbloodvesselat13days2)Extraembryonicbloodvesselsat17days2.Developmentofheart1)Positionandcardiactubeat22daysHeartbeatingat26days.2)Formationofheartloopat22-24days3)Formationofventricle4)Developmentofsinusvenosus3.Formationofcardiacsepta,valves,

arterialsystem,systemicveins

2weeksformationstart4weekscirculationstart8weeksfourchamberheartFormationofAVcanalFetalcirculationForamenOvaleClosureoccursintwostagesFunctionalclosureoccurswhenLApressurehigherthanRApressureThisisreversibleinthepresenceofhypoxemiaorhypovolemiaPermanentclosureoccursin5-7monthsForamenOvaleProbePatencyIspresentin50%ofchildren<5yearsold&inmorethan25%ofadultsIsapotentialavenueforairembolitoenterthesystemiccirculationApatentFOmaybebeneficialincertainheartmalformationsPatientswhorelyonthepatencyoftheforamenrequireaballoonatrialseptoplastyDuctusArteriosusClosureoccursintwostages80%Functionalclosureoccurs24hoursafterbirthThisisreversibleinthepresenceofhypoxemiaorhypovolemia90%Permanentclosureoccursin3months95%Permanentclosureoccursin6monthsFibrousconnectivetissueforms&permanentlysealsthelumenThisbecomestheligamentumarteriosumPersistentDuctusArteriosusThePDAinthepreterminfantisduetoaweakvasoconstrictorresponsetoO2andshouldbeconsideredanormalnotpathologicresponseThisPDAmaystillneedsurgicalcorrectionAlefttorightshuntthroughtheductuscanfloodthelungsoftheprematureinfantprolongingmechanicalventilation,eventuallyleadingtopulmonaryedema&rightsidedheartfailurePersistentDuctusArteriosusAPDAmayalsobebeneficialIncyanoticcongenitalheartmalformationswithrighttoleft&decreasedpulmonarybloodflowThePDAmaybethemajorroutebywhichthebloodreachesthepulmonaryarteriestoreceiveO2InthiscaseclosureoftheDAcausesseverecyanosis,tissuehypoxia&acidemiaTokeeptheductusopenpriortopalliativeorcorrectivesurgeryoftheheartmalformation,PGE1(0.05-0.1mcg/kg/min)canbeadministeredIVTohelpclosetheductuspriortosurgicalinterventiontoligatethePDA,Indomethacin(0.1-0.2mg/kg)canbeadministeredThisisaninhibitorofPGEsynthesisDiagnosticToolsHistoryandphysicalexaminationChestXray/EKG/BloodstudyEchocardiography/OtherimagingtoolsCatheterization/OtherinvasivetoolsAccuracyofToolsHistory/PE:important,rarelyspecificX-ray/EKG:not-confirmativeEchocardiography:confirmative,butnon-invasiveCardiaccatheterization:confirmative,butinvasiveOurroleaspediatriciansAttempttoprovideadefinitivediagnosisofaninnocentcardiacmurmuronthebasisofspecificclinicalfeatures,notjustasadiagnosisofexclusionProvidepatientsandfamilywithanexplanationandreassuranceCosteffectiveuseofmedicalresourcesPurposesofImagingAnatomic-pathologicdiagnosisHemodynamicassessment

(velocity,flow,pressure,stress-strain)Volume,function,wallmotion,torsionCoronaryperfusion/MetabolismTissuecharacterizationEchocardiographyEasy,non-invasive,accurate,real-timeAnatomicandphysiologicinformationChangedpracticeofpediatriccardiologyEchocardiography-ModalitiesM-mode/2-D/3-DDoppler/colorDopplerTrans-thoracic,trans-esophageal,trans-abdominal,trans-vaginal,intra-cardiac,intra-vascularEchocardiographyEchocardiographyM-modeEchocardiography

3-DEchocardiographyNewDevelopmentinEchoImaging:edgedetection/auto-measurementDoppler:3-Dflow/stress-strainContrastecho:coronaryperfusionFetalEcho:>16weeksPurposesofCatheterizationAnatomicdiagnosisHemodynamicassessmentInterventionalprocedureOtherImagingToolsMagneticResonanceImaging(MRI)CT/Electron-beamCT(EBCT)Radionuclide/SPECTPositronEmissionTomographyMagneticResonanceImaging

SpinechoGradientechoVelocityencoded

MagneticResonanceImagingSectionalstillimage/cineimage/3-DFlowinformation/volumeflowLesswindowdependant/post-opstudy,olderage/functionalevaluationComputerizedTomographyRadionuclideStudyRadionuclideStudyCongenitalHeartDiseaseInaggregate,congenitalheartdisease(CHD)comprisesarelativelylargepercentageofallmalformations.Theeffectonthepatientdependsontheseverityandtypeofthemalformation,butcanrangefromlethaltoclinicallyinsignificant.IntroductionPrevalence:Allbirths:1.56to7.7/1000Stillbirths:1to34.5/1000Livebirths:2.0to10.2/1000Affectedbymortality,diagnosticcriteria,lengthoffollow-up,etc.EtiologicBasisofCongenitalHeartDiseases1.Primarygeneticfactors

(10%)1)Chromosomal

5-10%2)Singlemutantgene

3%RecessiveDominant2.Genetic-environmentalinteraction(90%)1)Multifactorialinheritance,majority2)Riskstooffspringofanaffectedparent3)Environmentalcontribution