先天性心脏病(英文版)

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堵闭前心音图S1S2PDA堵闭后心音图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