1、Congenital Heart Disease(CHD)Yi Qi-jianProfessor&DirectorHeart Center,Childrens HospitalChongqing Medical UniversityChongqing 400014ChinaE-mail:QQ:1105643760 IntroductionCHD,the most common cardiac disease in CHD,the most common cardiac disease in childrenchildrenCritical time of embryoticCritical t
2、ime of embryotic cardiac 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 factorsGe
3、netic factors:single mutant genes/chromosomal Genetic factors:single mutant genes/chromosomal abnormalities/multifactorialabnormalities/multifactorial gene factors gene factorsEnvironmental factors:fetal environment/viral Environmental factors:fetal environment/viral infectioninfectionEtiology Class
4、ificationAccording 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)Ve
5、ntricular 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 o
6、f 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 SupracristalSupracristalperime
7、mbranousperimembranous：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 hy
8、pertrophyRV 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 sympto
9、msno 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 b
10、ackward,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 bilater
11、al 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
12、 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
13、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
14、: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 fluoroscop
15、y 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 no
16、t obviously large,mainly RV large aortic knob reducing2D Echocardiogram：ventricular septal echo discontinue，LALA、LV enlargementLV enlargement，ventricular ventricular septalseptal thicken and moving width augment thicken and moving width augmentColor Doppler Flow Image：VSD size and position，pressure
17、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 Angiocardiogra
18、phy 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 Exa
19、minationesCardiac 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 hear
20、t 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 occlusionAdaptationsAda
21、ptations：-perimembraous-perimembraous VSD 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 no right coronary Ao valve prolapse into VSD into VSD and aorta return and aorta return -r
22、esidual shunt after surgery -residual shunt after surgery Treatment For VSD Surgery Unsuitability percutaneousUnsuitability percutaneous occlusion occlusionmiddle-large VSD:infants who often middle-large VSD:infants who often suffered inflexible heart failure,suffered inflexible heart failure,pneumo
23、nia,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 Hemody
24、namicsClinical 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 h
25、ole ASD and coronary vein sinus(冠状静脉窦)ASD are fewer。Part of secondary hole ASDs can natural close(the rate1.5:1/Qs1.5:1）Systemic insufficientSystemic insufficient：pale,slimpale,slim，hypodynamiahypodynamia(乏乏力力)PA congestionPA congestion：cardiopalmuscardiopalmus(心悸心悸)and breath)and breath hard(hard(气
26、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 n
27、ormal,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 fixe
28、d splitingfixed 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 rsRappears rsR,conduction delay(,conduction delay(传导延传导延迟迟)ElectrocardiogramChes
29、t 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，“lu
30、ng hilumlung hilum dancing”phenomenon(dancing”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 incre
31、asedpulmonary circulation pulmonary circulation congestioncongestionEchocardiographyM-mode Echocardiogram:9898 RARA、RV RV enlargementenlargement，ventricular septel and LV posteroventricular septel and LV postero-wall moves in same waywall moves in same way2D Echocardiogram：atria septelatria septel d
32、iscontinue,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 pres
33、sure: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 com
34、plications,monitorcomplications,monitor PA pressure 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 1976AmplatzerAmplatzer two trays(t
35、wo trays(双盘双盘)ASD occluder)ASD occluder having been used from 1997having been used from 199780%ASD can be cured by occluder80%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
36、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 extensionASDthe maxmal atria septel extensionASD diameter diameter14mm14mmNo other malformation need surgeryNo other mal
37、formation 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
38、/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 montheshours,anatomic close time in 3
39、 monthes after birthafter birthIf the ductus continue open and have If the ductus continue open and have pathophysiologypathophysiology change change，be diagnosed CHD(PDA)be diagnosed CHD(PDA)Pathological anatomy 3 types:tube type funnel type window typePathophysiology and Hemodynamics Pathophysiolo
40、gy and HemodynamicsLA,LV hypervolemiaLA,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
41、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,acratiaincreasing,acratia(乏力乏力),and ca
42、rdiopalmus),and cardiopalmus(心心悸悸),short of breath(),short of breath(气喘气喘),cough after activity),cough after activity in 6 monthesin 6 monthes old.old.Most large PDAMost large PDA，repeatedly respiratory tract repeatedly respiratory tract infection(pneumonia)and CHF in infantinfection(pneumonia)and C
43、HF in infant Clinical ManifestationsPhysical examinationIn general:thin,thoracocyllosisIn general:thin,thoracocyllosis(胸廓畸形胸廓畸形),),differential cyanosedifferential cyanose （差异性青紫）（差异性青紫）(cyanose in (cyanose in lower limbsupper limbs,leftlower limbsupper limbs,leftright)in PAH(R to right)in PAH(R to
44、L shunt)L shunt)Heart:Heart:nLA,LV enlargement more or lessLA,LV enlargement more or lessnTypical sign:rough/loud/mechineryTypical sign:rough/loud/mechinery/continuous/continuous murmurmurmur in 2nd rib left border of sternum,and in 2nd rib left border of sternum,and conduct to left clavicle,neckcon
45、duct to left clavicle,neck and back;thrill 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 increasin
46、g,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 lar
47、ge 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 pumonaryshadow
48、of pumonary vessel thicken,lung field 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 bec
49、ome thin,lung field congestion may vessel become thin,lung field congestion may not obvious then,likenot obvious then,like deadwood(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 se
50、ptum 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 thro
