儿科学教学课件：Congenital Heart Disease

Congenital Heart Disease(CHD)IntroductionCHD,the most common cardiac disease in CHD,the most common cardiac disease in childrenchildrenCritical time of embryotic cardiac Critical time of embryotic cardiac development: development: second to eighth week of second to eighth week of gestationgestationIncidence:Incidence:7 78/10008/1000CHD result from interaction between genetic CHD result from interaction between genetic and environmental factorsand environmental factorsGenetic factors: single mutant genes/chromosomal Genetic factors: single mutant genes/chromosomal abnormalities/multifactorial gene factorsabnormalities/multifactorial gene factorsEnvironmental factors: fetal environment/viral Environmental factors: fetal environment/viral infectioninfectionEtiology ClassificationAccording to Hemodynamics:left-to-right shunts (without cyanosis) eg VSD,ASD,PDA,et alright-to-left shunts (with cyanosis) eg TOF,TGA,et alno shunt at all (without cyanosis) eg PS,AS,et alVentricular Septal Defect(VSD)Atria Septal Defect(ASD)Patent Ductus Arteriosus(PDA)Tetralogy of Fallot(TOF) Ventricular Septal Defect(VSD)Outline Incidence and rate of natural closurePathological anatomy Pathophysiology and HemodynamicsClinical ManifestationsAssistant ExaminationesElectrocardiogram/ /X-ray/ / Echocardiography/ / Cardiac Catheterization and AngiocardiographyComplicationsTreatmentIncidence and rate of natural closureVSD is the most common of all CHD, accounting for approximately 30 to 60% of all full-term newborn with CHD.Most tiny muscular and perimembranous defects（0.5cm）have high chance of spontaneous closure within 6 to 12 months after birth.Pathological anatomy SupracristalSupracristalperimembranousperimembranous：60-70%60-70%Muscular defectMuscular defect： Pathophysiology and HemodynamicsSmall defect(diameter 1.0cm)，shunt/pulmonary flow 60，LV,RV enlargement，pulmonary hypertension double shunt or R to L，finally , Eisenmengers syndrom Pathophysiology and HemodynamicsLA,LV enlargement and hypertrophyRV flow increased、enlargement、hypertrophyPulmonary circulation flow increased Systemic circulation flow insufficiencyClinical ManifestationsTwo determinants for clinical manifestation of isolated VSD size of defect volume of shunt Clinical ManifestationsSmall VSD: Small VSD: no obvious symptomsno obvious symptomsModerate VSD:Moderate VSD: Symptoms: :shortnessshortness of breath after crying of breath after crying or sucking in infant; or sucking in infant; dyspnea after exercise,heart- dyspnea after exercise,heart- throb( throb(心悸心悸),chest distress, growing ),chest distress, growing development backward,and development backward,and respiratory tract infection respiratory tract infection repeatedly in the senior. repeatedly in the senior.Clinical ManifestationsSigns inspection: precordium full, apex beat diffusion palpation: systolic thrill（震颤）（震颤） in L3-4 percuss: heart boundary expand in bilateral auscultation: 3-4/6 systolic murmur in L3-4 P2 strengthen/hyperfunction Clinical Manifestations Large VSD with pulmonary resistance increased Symptoms: : cyanosis after exercise or continue cyanosis, sporadic hemoptysis(喀血）喀血）, clubbing, growth development backward obviously Clinical ManifestationsSigns inspection: precordium hunch(隆起隆起), apex beat diffusion palpation: systolic thrill in L3-4 percuss: heart boundary obviously expand in bilateral auscultation: 3-4/6 brief systolic murmur in L3-4,P2 strengthen/hyperfunctionSmall VSD: normal or mild LV hypertrophyMorderate-large VSD: LV hypertrophy or both ventricular hypertrophyPA pressure obviously increased: mainly RV hypertrophy Electrocardiogram(ECG)Assistant ExaminationesSmall VSD: normal or mild LV hypertrophyMorderate-large VSD: LV hypertrophy or both ventricular hypertrophyPA pressure obviously increased: mainly RV hypertrophyChest X-raySmall VSD: pulmonary flow slightly increased normal in lung hilar shadow PA segment normal or mild heave heart shape normal or mild large, CTR 0.5-0.55 aortic knob(主动脉结) reducing Chest X-rayMorderate-large VSD: pulmonary flow obviously increased lung hilar shadow augment, thicken, hilar dancing in chest fluoroscopy PA segment heave heart shape obviously large, CTR 0.56-0.7, both ventricular large, mainly in LV, aortic knob reducingChest X-rayPA pressure seriously increased: pulmonary circulatory flow lower PA segment seriously heave lung artery rough, twist, discontinue, like rat tail or residual root heart shape not obviously large, mainly RV large aortic knob reducing2D Echocardiogram ：ventricular septal echo discontinue，LALA、LV enlargementLV enlargement，ventricular ventricular septal thicken and moving width augmentseptal thicken and moving width augmentColor Doppler Flow Image：VSD size and position，pressure difference(pressure difference(压差压差) between LV and RV) between LV and RV，pulmonary pressure, RV pressurepulmonary pressure, RV pressure、lung lung resistance and shunt flow can be takenresistance and shunt flow can be takenEchocardiographyAssistant ExaminationesCardiac Catheterization and Angiocardiography Right cardiac catheterizationRight cardiac catheterization：Exceptional channelExceptional channel：RV LVRV LVBlood oxygen dataBlood oxygen data：RV oxygen saturation RV oxygen saturation (SO2,(SO2,氧饱和度氧饱和度)RASO2)RASO2Pressure dataPressure data：RV pressure increasedRV pressure increasedAssistant ExaminationesCardiac Catheterization and AngiocardiographyWhat can be found What can be found in left cardiac in left cardiac catheterization? catheterization? What can be What can be observed in LV observed in LV angiocardiography? angiocardiography? Complications(Left Right shunt)PneumoniaCongestive heart failurePulmonary artery hypertensionInfection endocarditisTreatment For VSD Internal medical treatmentInternal medical treatment prevent and treat all kinds of complications, prevent and treat all kinds of complications,Interventional cardiac catheterization percutaneous VSD occlusionAdaptationsAdaptations： -perimembraous VSD-perimembraous VSD -age2 -age2岁岁 - -alone VSDalone VSD VSD upper edge distance to right Ao VSD upper edge distance to right Ao valve2.0mm valve2.0mm no right coronary Ao valve prolapse into VSD no right coronary Ao valve prolapse into VSD and aorta return and aorta return - residual shunt after surgery - residual shunt after surgery Treatment For VSD Surgery Unsuitability percutaneous occlusionUnsuitability percutaneous occlusionmiddle-large VSD: infants who often middle-large VSD: infants who often suffered inflexible heart failure, suffered inflexible heart failure, pneumonia, growing backward and pulmonary pneumonia, growing backward and pulmonary hypertension should be operated early in 6 hypertension should be operated early in 6 months old.months old.Atrial Septal Defect （ASD）OutlineIncidence and rate of natural closurePathological anatomy Pathophysiology and HemodynamicsClinical ManifestationsAssistant ExaminationesElectrocardiogram/X-ray/ Echocardiography/ Cardiac Catheterization and AngiocardiographyComplicationsTreatmentIncidence and rate of natural closureASD is one of common CHD in childrenIncidence: 20-30% of CHDSecondary hole ASD is the most type，primary hole ASD and coronary vein sinus(冠状静脉窦)ASD are fewer。Part of secondary hole ASDs can natural close(the rate1.5:1Qp/Qs1.5:1）Systemic insufficientSystemic insufficient：pale, slimpale, slim，hypodynamia(hypodynamia(乏乏力力) )PA congestionPA congestion：cardiopalmus(cardiopalmus(心悸心悸)and breath )and breath hard(hard(气短气短) after exercising, temporary cyanose, ) after exercising, temporary cyanose, respiratory tract infectionrespiratory tract infectionInfant, may have not symptom, often be found in Infant, may have not symptom, often be found in physical examinationphysical examinationClinical ManifestationsSignsSigns Most normal, no cyanoseMost normal, no cyanoseHeart examination Heart examination Right side expand more or lessRight side expand more or lessNo thrill(No thrill(震颤震颤) )23236 systolic murmur in 23 rib left 6 systolic murmur in 23 rib left along breastbonealong breastboneP P2 2 strengthenstrengthen，S S2 2 fixed spliting(fixed spliting(固定分裂固定分裂) )Assistant ExaminationesElectrocardiography Major featuresElectrical axis inclined to right(Electrical axis inclined to right(电轴右偏电轴右偏) )RA enlargementRA enlargementLead VLead Vl l appears rsR, conduction delay(appears rsR, conduction delay(传导延传导延迟迟) )ElectrocardiogramChest X-RaySmall ASD: normalSmall ASD: normalLarge ASD, large shunt:Large ASD, large shunt:Vascular shadow in both lungs increasedVascular shadow in both lungs increased，pulmonary circulation engorgedpulmonary circulation engorgedSegment of pulmonary artery prominentSegment of pulmonary artery prominent， “lung hilum dancinglung hilum dancing” phenomenon( phenomenon(肺门舞蹈肺门舞蹈) ) RARA、RV enlargementRV enlargementAorta knot lessAorta knot less（主动脉结缩小）（主动脉结缩小）CTR increasedCTR increased（心胸比例）（心胸比例）The diagrams of chest roentgenograms of ASDvascular shadow in both vascular shadow in both lungs increasedlungs increasedpulmonary circulation pulmonary circulation congestioncongestionEchocardiographyM-mode Echocardiogram: 9898 RARA、RV RV enlargementenlargement，ventricular septel and LV postero-ventricular septel and LV postero-wall moves in same waywall moves in same way2D Echocardiogram ：atria septel atria septel discontinue, the end like match-stickdiscontinue, the end like match-stickColor Doppler Flow Image：multi-colored multi-colored flow shunt from LA to RA through ASDflow shunt from LA to RA through ASDCardiac CatheterizationRight cardiac catheterization (1)data of blood oxygen: RA SO2SVC,IVC(2)data of pressure: RA、RV、PA(3)abnormal channel：RA to LA,PVComplications(Left Right shunt)PneumoniaCongestive heart failurePulmonary artery hypertensionInfection endocarditis: fewerTreatment for ASDInternal medical treatmentInternal medical treatment prevent and treat all kinds of prevent and treat all kinds of complications,monitor PA pressurecomplications,monitor PA pressureInterventional therapy T Transcatheter ASD occlusion It is an important technical renovation in It is an important technical renovation in medical sciencemedical scienceBeginning from 1976Beginning from 1976Amplatzer two trays(Amplatzer two trays(双盘双盘) ASD occluder ) ASD occluder having been used from 1997having been used from 199780% ASD can be cured by occluder 80% ASD can be cured by occluder Adaptations age2 years oldage2 years olddiameter 4-36mm,secondary central ASDdiameter 4-36mm,secondary central ASDthe distance of defect edge to coronary vein the distance of defect edge to coronary vein sinus(sinus(冠状静脉窦冠状静脉窦) )，SVCSVC、IVC and PV5mm,to IVC and PV5mm,to chamber valve7mmchamber valve7mmthe maxmal atria septel extensionASD diameterthe maxmal atria septel extensionASD diameter14mm14mmNo other malformation need surgeryNo other malformation need surgery Treatment for ASDsurgery Large ASDASDs that can not treat by occlusionPatent Ductus Arteriosus （PDA） OutlineIncidence and rate of natural closurePathological anatomy Pathophysiology and HemodynamicsClinical ManifestationsAssistant ExaminesElectrocardiogram/ /X-ray/ / Echocardiography/ / Cardiac Catheterization and AngiocardiographyComplicationsTreatmentIncidence and rate of natural closureIncidence:15Incidence:15 in CHDin CHDThe ductus functionality close after born 15 The ductus functionality close after born 15 hours, anatomic close time in 3 monthes hours, anatomic close time in 3 monthes after birthafter birthIf the ductus continue open and have If the ductus continue open and have pathophysiology changepathophysiology change，be diagnosed CHD(PDA)be diagnosed CHD(PDA)Pathological anatomy 3 types:tube type funnel type window typePathophysiology and Hemodynamics Pathophysiology and HemodynamicsLA ,LV hypervolemia(LA ,LV hypervolemia(血容量增加血容量增加),enlargement),enlargement，hypertrophyhypertrophyPA engorgement(PA engorgement(充血充血) )Systemic circulation blood-supply insufficient Systemic circulation blood-supply insufficient ( (供血不足供血不足) )Peripheral artery diastolic falling(Peripheral artery diastolic falling(舒舒张压力下张压力下降降) )，(pulse pressure broadening(pulse pressure broadening(脉压增宽脉压增宽 ) ) Clinical Manifestations Symptoms Small PDASmall PDA，symptomlesssymptomlessMiddle and large PDAMiddle and large PDA，respiratory rate respiratory rate increasing, acratia(increasing, acratia(乏力乏力),and cardiopalmus(),and cardiopalmus(心心悸悸),short of breath(),short of breath(气喘气喘),cough after activity ),cough after activity in 6 monthes old.in 6 monthes old.Most large PDAMost large PDA，repeatedly respiratory tract repeatedly respiratory tract infection (pneumonia) and CHF in infantinfection (pneumonia) and CHF in infant Clinical ManifestationsPhysical examinationIn general: thin, thoracocyllosis(In general: thin, thoracocyllosis(胸廓畸形胸廓畸形), ), differential cyanose differential cyanose （差异性青紫）（差异性青紫）(cyanose in (cyanose in lower limbsupper limbs,leftright) in PAH (R to lower limbsupper limbs,leftright) in PAH (R to L shunt)L shunt)Heart:Heart: nLA,LV enlargement more or lessLA,LV enlargement more or lessnTypical sign: rough/loud/mechinery/continuous Typical sign: rough/loud/mechinery/continuous murmurmurmur in 2nd rib left border of sternum, and in 2nd rib left border of sternum, and conduct to left clavicle,neck and back; thrill conduct to left clavicle,neck and back; thrill nP2 strengthenP2 strengthenClinical ManifestationsPeripheral blood vessel sign:Peripheral blood vessel sign: systemic systemic circulation diastolic pressure dropping, circulation diastolic pressure dropping, pulse pressure difference increasing, just pulse pressure difference increasing, just like the sign of aortic valve insufficiencylike the sign of aortic valve insufficiencynWater hammer pulse(Water hammer pulse(水冲脉水冲脉) )nSign of capillary pulsation(Sign of capillary pulsation(毛细血管搏动征毛细血管搏动征) )nPeripheral large artery gunshot(Peripheral large artery gunshot(周围大动周围大动脉枪击声脉枪击声) )Assistant ExaminationesElectrocardiography: LV hypertrophy Chest Radiography Small PDA: normalSmall PDA: normalComparatively large PDA:Comparatively large PDA: LA,LV enlargement, segment of PA prominent, LA,LV enlargement, segment of PA prominent, shadow of pumonary vessel thicken, lung field shadow of pumonary vessel thicken, lung field congestion, aorta broadening.congestion, aorta broadening.Large PDA and PAH: LV,RV enlargement, obvious Large PDA and PAH: LV,RV enlargement, obvious for RV, segment of PA prominent, periphery for RV, segment of PA prominent, periphery vessel become thin, lung field congestion may vessel become thin, lung field congestion may not obvious then,like deadwood(not obvious then,like deadwood(枯枝或截枝状枯枝或截枝状) )EchocardiographyM-mode Echocardiogram: LV、LA enlargement， aorta anterior-posterior augmentation，the activity range of LV backwall and ventricular septum increasing2D Echocardiogram ：direct indication PDA between MPA and descending aortaColor Doppler Flow Image：red shunt from DAO to MPA through PDACardiac Catheterization and AngiocardiographyRight cardiac cathterization： Abnormal wayAbnormal way：catheter from PA to DAO catheter from PA to DAO through PDA directly through PDA directly data of blood oxygen data of blood oxygen：PARVPARV，means?means? data of pressure data of pressure：monitor PA pressure monitor PA pressure and wedge pressure PA( and wedge pressure PA(肺小动脉契压肺小动脉契压) )，and and calculate total pulmonary resistance and calculate total pulmonary resistance and pulmonary small artery resistance pulmonary small artery resistanceCardiac Catheterization and AngiocardiographyAorta angiocardiography ascending aorta and aortic arch enlargement ascending aorta and aortic arch enlargement， PA and arterial duct developing(PA and arterial duct developing(显影显影) at the ) at the same timesame time，and measurability of diameter,and and measurability of diameter,and shape of duct shape of ductComplications (Left to Right shunt)PneumoniaCongestive heart failurePulmonary artery hypertensionInfection endocarditis: fewerTreatment For PDA Internal medicine to prevent and treat complicationsTreatment For PDA Interventional therapy,transcatheter PDA occlusion,first choice at present Coil(spring coil)Coil(spring coil)：PDA narrowest2.0mm / weight4kg / age6mon / PDA narrowest2 ):many happen in senior(2 years old) and adultyears old) and adult。It should be considered It should be considered in TOF with fever,headache, vomit, hypersomnia in TOF with fever,headache, vomit, hypersomnia ( (嗜睡嗜睡), convulsion(), convulsion(抽搐抽搐), hemiplegy(), hemiplegy(偏瘫偏瘫) )Infective endocarditis:Infective endocarditis: TOF less pneumonia and HFTOF less pneumonia and HF TreatmentInternal medicine treatmentActivity is restricted Activity is restricted Prevent infectionPrevent infectionIntake enough water everyday to prevent Intake enough water everyday to prevent dehydration(dehydration(脱水脱水) )Prevent inducements of cyanotic spells such Prevent inducements of cyanotic spells such as fever, iron deficiency anemiaas fever, iron deficiency anemiaTreatmentPrevent and control cyanotic spells： Chest-knee position at once Morphine: 0.10.2mgkg，im，or propranolol: O.1mgkg iv，injection for 510 min prevention: propranolol 1mgkgd,oralTreatmentSurgery70% operated once within 1 years old70% operated once within 1 years oldPalliative surgery(Palliative surgery(姑息性手术姑息性手术 ) should be ) should be considered in young baby, serious, poor considered in young baby, serious, poor development of PA branch, hypoplastic LV development of PA branch, hypoplastic LV Differentiation of common CHDtypesVSDASDPDATOFclassificationL to R shuntR to L shuntsignMurmur positionMurmur loundness & propertythrill thrill P2L 3-4L 2-3L 2L 2-32-5/6 2-5/6 rough SM rough SM transmit transmit widewide2-3/6 2-3/6 blowy SMblowy SMtransmit transmit narrownarrow2-4/6 2-4/6 continuouscontinuousmachine machine transmit wide transmit wide2-4/6 2-4/6 spray SM spray SM transmit transmit widewideyesNoyesMay be yeshyperfunctionFixed splithyperfunctionreduceDifferentiation of common CHDtypesVSDASDPDATOFclassificationL to R shuntR to L shuntX- rayA or V A or V enlargementenlargementPA segmentPA segmentlung field lung field Porta pulmonis(肺门舞蹈)LV,LALV,LARVRVRA,RVLA,LVRVRVhyperhyperProtrude (凸出)protrudeprotrudeprotrudeprotrudeSunkenSunken( (凹陷凹陷) )Congestion(充血充血)congestioncongestionLack blood(少血少血)YesYesYesNoEisenmenger syndromeThe term “eisenmenger syndrome” is used here for the combination of pulmonary hypertention with reversed shunt through either a VSD,ASD or PDA(or other communications between the aorta and lesser circulation).ReferencesYang Siyuan，Pediatric cardiology，People Health PressZhu Futang, Practical pediatrics, People Health PressRudolph CD,Rudolph AM,Hostetter MK,et al.Rudolphs Pediatrics. People Health PressPlease attention to（Command）ASD,VSD,PDA,TOFPathologic anatomy and hemodynamicsClinic manifestationComplicationsPrinciple of diagnosis and treatmentThank you for your attention!