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CongenitalHeartDiseaseFatusCordisBloodCirculation
FetalCirculation1.Placentaistheplacetochangenutritionandgas;2.Resistanceofpulmonarycirculationishigherthansystemiccirculation;3.Patentductusarteriosus
;4.Acleistocardia;5.Mixedbloodintheheart.NeonatalCirculation
1. Presureofpulmonarycirculationdescended;2. Foraminaovaleoccludedfossaeovalis;3. Ductusarteriosusoccludedchordaductusarteriosi;4. Therearen’tanymixedbloodintheheart.Etiology
1.Endopathic:Heredity;Chromosomeaberration;2.Exopathic:Intrauterineinfection;Contactedradioactiveray;Disease;ClassificationofCHD
1.Left-to-rightshunts:VSD;ASD;PDA.2.Right-to-leftshunts:1).Pulmonaryischemic:TOF,EisenmengerSyndrome.2).PulmonaryHypostasis:Completetranspositionofgreatarteries;3.Noshunts:Pulmonarystenosis;Aorticstenosis;Dextrocardia.
Diagnosis
1.History:Cyophorichistoryofhismother,symptoms,ageofonset;2.Physicalexamination:3.Specialinspection:X—ray,EKG,UCG,MRI,Angiocardiography,Cardiaccatheterization.Diagnosis
SegmentalDiagnosis:VanPraagh
VentricularSeptalDefect(VSD)Hemodynamics
BeforePulmonaryHypertension
AfterPulmonaryHypertensionClinicalManifestationsSmalldefect(maladiedeRoger):<1/3ofcrosssectionofaortaClinicalManifestationsModeratedefect(limitedshunts):1/3—2/3ofcrosssectionofaortaClinicalManifestationsLargerdect(infiniteshunts):>2/3ofcrosssectionofaortaClinicalManifestations1.VSDwithlargeL—Rshunts:Pulmonarytosystemicflowratiosmorethan3.0:1.ClinicalManifestationsa.Hypotrophy,Dysplasia,poorweightgain
,b.poorfeeding.c.Hypodynamia,Anhelation,Hyperhidrosis.d.Pneumonia,Heartfailure.ClinicalManifestations
e.Cerchnus
f.Physicalsignsg.X—rayh.ECGi.UCGj.MRIk.CardiaccatheterizationClinicalManifestations2.VSDwithhighpulmonaryvascularresistance:pulmonarysystemicresistanceratiosof40—70%ClinicalManifestations
a. Hypotrophy,dysplasiab. Chickenbreastc. Rightventricularhypertrophyd.Shorterofsystolicmurmur,nothrill,themiddiastolicmurmurdisappearClinicalManifestationse.X—raymayshowanormalcardiacconfigurationandareductioninpulmonaryvascularmarkingsf.RightventricularhypertrophymaybeobservedontheECGClinicalManifestations3.Eisenmenger’sSyndrome:thepulmonaryarterialpressureisatornearsystemiclevel,andtheshuntsiseitherbidirectionalorR—LClinicalManifestationsTreatment
1.Medicaltreatment:theneedforantibioticprophylaxisandacuteawarenessofthepossibilityofinfectiveendocarditisareimportantpointstorememberintreatingpatientswithVSD.
2.SurgicalTreatment:a.Moderateandsmalldefectb.Largershunts3.InterventionaltherapyTreatment
AtrialSeptalDefect(ASD)TheClinicalSignificanceofASD1.ASDisoneofthemostcommonlycongenitalcardiacanomaliesinadultsbutisveryrarelydiagnosedandevenlesscommonlyresultsindisabilityininfants.
2.ThediagnosisofASDisn’tdifficulty.Operationideallyinpatientshouldbeadvisedforallpatientswhentheyhaven’tgonetoshool.TheClinicalSignificanceofASD3.ASDisacommonassociatedlesioninothercongenitalheartdisease.TheClinicalSignificanceofASDFormationofAtrialSeptumHemodynamicsManifestations
Symptoms:AbouthalfofpatientswithASDareasymptomaticwhenthediagnosisismade.Symptomsdevelopin60%ofpatientsbyage30.1.Dyspneaonexertion(65%);2.Palpitation(20%):duetoatrialarrhythmia.3.Edema,ascites:duetorightheartfailure.Manifestations
Signs:Thephysicalsignsdependonthemagnitudeofpulmonarybloodflowandthestatusofpulmonaryvascularresistance.Manifestations1.VisibleandPalpablepulseinthesecondorthirdleftintercostalspace.2.Thesecondheartsoundiswidelyandpexicsplit.Manifestations3.Pulmonarysystolicejectionmurmurisalmostalwayspresentbecauseofincreasedflowthroughthepulmonaryvalve,butthemurmurisoftensoft.Manifestations4.Othermurmurs:Middiastolicmurmur:duetorelativetricuspidstenosis.Earlydiastolicmurmur:duetopulmonaryinsufficiency.ManifestationsX—ray
1.Rightatriumandrightventricleenlarged.2.Pulmonaryplethorawithincreasedlungmarking.3.Radioscopyshows“hilardance”.ECG
1.Rightventricularhypertrophy;2.Rightbundlebranchblock.
MRICardiaccatheterizationAngiocardiographyComplications
1. PulmonaryvasculardiseaseAcquiredEisenmenger’sSyndrome.2. HeartFailure3. AtrialFibfillation4. InfectionendocarditisPrognosis
TheprognosisinASDisgoodevenwithoutsurgicaltreatment,andifanoperationisperformedbeforesymptomsdevelop,thepatientshouldhaveanormallifeexpectancy.InterventionaltherapyTreatment
PatentDuctusArteriousus(PDA)Typing1. Tubifom2. Choanoid
3. FenestrateHemodynamicsManifestations
Symptoms(largeshunts):Beforepulmonaryhypertension:HeartfailureAfterpulmonaryhypertension:DyspneaonexertionDifferentialcyanosisSigns:“machinery”murmur
ManifestationsS1S2PDA堵閉前心音圖S1S2PDA堵閉后心音圖ManifestationsManifestationsLargeshunts:cardiacenlargementwidepulsepressuremiddiastolicmurmur
TheECGisofnormalwhentheshuntissmall(about20%),butisbiventricularhypertrophyinlargeleft-to-rightshuntswithpulmonaryhypertension.ECG
pulmonaryplethorawithlefeatrialandleftventricularenlargedandalargeascendingaortaareseeninpatientswithlargeshunts.X—rayUCG
It’sindicatedtoconfirmthepresenceofaleft-to-rightshuntatpulmonaryarteriallevelandtomeasurethepulmonaryandsystemicpressureandflow.CardiacCatheterizationAninjectionofcontrastmaterialintotheaorticarchwiththepatientintheleftanteriorobliquepositionwillfillthepulmonaryarteryandoutlineductus.AngiocardiographyTreatment
SurgicalTreatmentPremature—Indocin:first—0.2mg/kgsecondandthird—postnatal<48hrs:0.1mg/kg2—7days:0.2mg/kgq12h>8days:0.25mg/kgTreatmentInterventionaltherapyTetralogyofFallot(TOF)PathologicalAnatomy
1.Rightventricularoutflowobstruction2.VSD3.Overridingaorta4.RightventricularhypertrophyF3=PS+RVH+ASDF5=F4+ASDHemodynamicsManifestations
Symptoms
1.Cyanosis
ClubbingoffingersManifestations
2.Attacksoffaintness
3.Squatting
Signs
1.Rightventricularheave2.Intensityandlengthofthemurmur3.PulmonaryvalvesoundisusuallyinaudibleManifestations
X—ray
Thecombinationofrightventricularhypertrophyandinfundibularstenosisgivestheclassic“coeurensabot”(boot-shapedheart)radiologicalpicture,withtheapexpointingupwardandtotheleft.ECG
TheECGalwaysshowssomeevidenceofrightventricularhypertrophyinTOF,butthechangesmaybesurprisinglymild.UCG
Itispossibletodeterminethepresenceandextentofoverridingoftheaortaandtoseethebreakincontinuityoftheechoesthatrepresentstheventricularseptaldefect.Dopplerstudiescandetectthedisturbanceofflowassociatedwithpulmonaryvalveorinfundibularstenosis.CardiacCatheterization
It’sindicatedbeforeanysurgicaloperationinpatientswithTOF.Theaimofthestudyist
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