儿科学教学课件：07-CHD（先天性心脏病）

1、Congenital Heart Disease(CHD)IntroductionCHD,the most common cardiac disease in childrenCritical time of embryotic cardiac development: second to eighth week of gestationIncidence:78/1000CHD result from interaction between genetic and environmental factorsGenetic factors: single mutant genes/chromos

2、omal abnormalities/multifactorial gene factorsEnvironmental factors: fetal environment/viral infectionEtiology 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) e

3、g 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 ExaminationesElect

4、rocardiogram/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（0.5c

5、m）have high chance of spontaneous closure within 6 to 12 months after birth.Pathological anatomy Supracristalperimembranous：60-70%Muscular defect： Pathophysiology and HemodynamicsSmall defect(diameter 1.0cm)，shunt/pulmonary flow 60，LV,RV enlargement，pulmonary hypertension double shunt or R to L，fina

6、lly , 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

7、defect volume of shunt Clinical ManifestationsSmall VSD: no obvious symptomsModerate VSD: Symptoms:shortness of breath after crying or sucking in infant; dyspnea after exercise,heart- throb(心悸),chest distress, growing development backward,and respiratory tract infection repeatedly in the senior.Clin

8、ical ManifestationsSigns inspection: precordium full, apex beat diffusion palpation: systolic thrill（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 S

9、ymptoms: cyanosis after exercise or continue cyanosis, sporadic hemoptysis(喀血）, clubbing, growth development backward obviously Clinical ManifestationsSigns inspection: precordium hunch(隆起), apex beat diffusion palpation: systolic thrill in L3-4 percuss: heart boundary obviously expand in bilateral

10、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: norma

11、l 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

12、.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-r

13、ayPA 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，LA、LV enlar

14、gement，ventricular septal thicken and moving width augmentColor Doppler Flow Image：VSD size and position，pressure difference(压差) between LV and RV，pulmonary pressure, RV pressure、lung resistance and shunt flow can be takenEchocardiographyAssistant ExaminationesCardiac Catheterization and Angiocardio

15、graphy Right cardiac catheterization：Exceptional channel：RV LVBlood oxygen data：RV oxygen saturation (SO2,氧饱和度)RASO2Pressure data：RV pressure increasedAssistant ExaminationesCardiac Catheterization and AngiocardiographyWhat can be found in left cardiac catheterization? What can be observed in LV ang

16、iocardiography? Complications(Left Right shunt)PneumoniaCongestive heart failurePulmonary artery hypertensionInfection endocarditisTreatment For VSD Internal medical treatment prevent and treat all kinds of complications,Interventional cardiac catheterization percutaneous VSD occlusionAdaptations： -

17、perimembraous VSD -age2岁 -alone VSD VSD upper edge distance to right Ao valve2.0mm no right coronary Ao valve prolapse into VSD and aorta return - residual shunt after surgery Treatment For VSD Surgery Unsuitability percutaneous occlusionmiddle-large VSD: infants who often suffered inflexible heart

18、failure, pneumonia, growing backward and pulmonary hypertension should be operated early in 6 months old.Atrial Septal Defect （ASD）OutlineIncidence and rate of natural closurePathological anatomy Pathophysiology and HemodynamicsClinical ManifestationsAssistant ExaminationesElectrocardiogram/X-ray/ E

19、chocardiography/ 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

20、 ASDs can natural close(the rate1.5:1）Systemic insufficient：pale, slim，hypodynamia(乏力)PA congestion：cardiopalmus(心悸)and breath hard(气短) after exercising, temporary cyanose, respiratory tract infectionInfant, may have not symptom, often be found in physical examinationClinical ManifestationsSigns Mos

21、t normal, no cyanoseHeart examination Right side expand more or lessNo thrill(震颤)236 systolic murmur in 23 rib left along breastboneP2 strengthen，S2 fixed spliting(固定分裂)Assistant ExaminationesElectrocardiography Major featuresElectrical axis inclined to right(电轴右偏)RA enlargementLead Vl appears rsR,

22、conduction delay(传导延迟)ElectrocardiogramChest X-RaySmall ASD: normalLarge ASD, large shunt:Vascular shadow in both lungs increased，pulmonary circulation engorgedSegment of pulmonary artery prominent， “lung hilum dancing” phenomenon(肺门舞蹈) RA、RV enlargementAorta knot less（主动脉结缩小）CTR increased（心胸比例）The

23、diagrams of chest roentgenograms of ASDvascular shadow in both lungs increasedpulmonary circulation congestionEchocardiographyM-mode Echocardiogram: 98 RA、RV enlargement，ventricular septel and LV postero-wall moves in same way2D Echocardiogram ：atria septel discontinue, the end like match-stickColor

24、 Doppler Flow Image：multi-colored flow 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 arter

25、y hypertensionInfection endocarditis: fewerTreatment for ASDInternal medical treatment prevent and treat all kinds of complications,monitor PA pressureInterventional therapy Transcatheter ASD occlusion It is an important technical renovation in medical scienceBeginning from 1976Amplatzer two trays(双

26、盘) ASD occluder having been used from 199780% ASD can be cured by occluder Adaptations age2 years olddiameter 4-36mm,secondary central ASDthe distance of defect edge to coronary vein sinus(冠状静脉窦)，SVC、IVC and PV5mm,to chamber valve7mmthe maxmal atria septel extensionASD diameter14mmNo other malformat

27、ion need surgery Process of transcathter ASD occlusion use Amplatzer Occluder Treatment for ASDsurgery Large ASDASDs that can not treat by occlusionPatent Ductus Arteriosus （PDA） OutlineIncidence and rate of natural closurePathological anatomy Pathophysiology and HemodynamicsClinical ManifestationsA

28、ssistant ExaminesElectrocardiogram/X-ray/ Echocardiography/ Cardiac Catheterization and AngiocardiographyComplicationsTreatmentIncidence and rate of natural closureIncidence:15 in CHDThe ductus functionality close after born 15 hours, anatomic close time in 3 monthes after birthIf the ductus continu

29、e open and have pathophysiology change，be diagnosed CHD(PDA)Pathological anatomy 3 types:tube type funnel type window typePathophysiology and Hemodynamics Pathophysiology and HemodynamicsLA ,LV hypervolemia(血容量增加),enlargement，hypertrophyPA engorgement(充血)Systemic circulation blood-supply insufficien

30、t (供血不足)Peripheral artery diastolic falling(舒张压力下降)，(pulse pressure broadening(脉压增宽 ) Clinical Manifestations Symptoms Small PDA，symptomlessMiddle and large PDA，respiratory rate increasing, acratia(乏力),and cardiopalmus(心悸),short of breath(气喘),cough after activity in 6 monthes old.Most large PDA，repe

31、atedly respiratory tract infection (pneumonia) and CHF in infant Clinical ManifestationsPhysical examinationIn general: thin, thoracocyllosis(胸廓畸形), differential cyanose （差异性青紫）(cyanose in lower limbsupper limbs,leftright) in PAH (R to L shunt)Heart: LA,LV enlargement more or lessTypical sign: rough

32、/loud/mechinery/continuous murmur in 2nd rib left border of sternum, and conduct to left clavicle,neck and back; thrill P2 strengthenClinical ManifestationsPeripheral blood vessel sign: systemic circulation diastolic pressure dropping, pulse pressure difference increasing, just like the sign of aort

33、ic valve insufficiencyWater hammer pulse(水冲脉)Sign of capillary pulsation(毛细血管搏动征)Peripheral large artery gunshot(周围大动脉枪击声)Assistant ExaminationesElectrocardiography: LV hypertrophy Chest Radiography Small PDA: normalComparatively large PDA: LA,LV enlargement, segment of PA prominent, shadow of pumon

34、ary vessel thicken, lung field congestion, aorta broadening.Large PDA and PAH: LV,RV enlargement, obvious for RV, segment of PA prominent, periphery vessel become thin, lung field congestion may not obvious then,like deadwood(枯枝或截枝状)EchocardiographyM-mode Echocardiogram: LV、LA enlargement， aorta ant

35、erior-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 cathteriza

36、tion： Abnormal way：catheter from PA to DAO through PDA directly data of blood oxygen：PARV，means? data of pressure：monitor PA pressure and wedge pressure PA(肺小动脉契压)，and calculate total pulmonary resistance and pulmonary small artery resistanceCardiac Catheterization and AngiocardiographyAorta angioca

37、rdiography ascending aorta and aortic arch enlargement， PA and arterial duct developing(显影) at the same time，and measurability of diameter,and shape of ductComplications (Left to Right shunt)PneumoniaCongestive heart failurePulmonary artery hypertensionInfection endocarditis: fewerTreatment For PDA

38、Internal medicine to prevent and treat complicationsTreatment For PDA Interventional therapy,transcatheter PDA occlusion,first choice at present Coil(spring coil)：PDA narrowest2 years old) and adult。It should be considered in TOF with fever,headache, vomit, hypersomnia (嗜睡), convulsion(抽搐), hemipleg

39、y(偏瘫)Infective endocarditis: TOF less pneumonia and HF TreatmentInternal medicine treatmentActivity is restricted Prevent infectionIntake enough water everyday to prevent dehydration(脱水)Prevent inducements of cyanotic spells such as 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 oldPalliati