儿科教学 儿科CHD.ppt

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1、 先天性心脏病先天性心脏病先天性心脏病先天性心脏病 Congenital Heart DiseasesCongenital Heart Diseases山东大学齐鲁医院山东大学齐鲁医院儿童医疗中心心内科儿童医疗中心心内科夏夏 伟伟出生后血压的变化出生后血压的变化n出生后出生后24h6540mmHgn1岁岁8550mmHgn4岁岁9050mmHgn6岁岁9460mmHg Blood Pressure 血压血压摘引自福州军区总医院儿科资料年龄分组年龄分组收缩压收缩压舒张压舒张压7103.510.069.910.09105.110.369.410.211105.713.270.810.913107.

2、912.469.59.4身高增长身高增长10cm，血压升高，血压升高33.522.5mmHg体重增加体重增加4kg，血压升高，血压升高2.5312mmHg 年龄增长年龄增长1岁，血压升高岁，血压升高22.51.01.5mmHg摘自广东省农村3826名儿童血压普查资料CHD发病情况发病情况n68（活产新生儿）（活产新生儿）n15万年（新病人）万年（新病人）n先心病占所有先天畸形的28%。n根据WHO提供的资料，全球每年有150万儿童出生时患有先心病。n美国国家卫生统计中心统计，1988-1990年间有30余万1岁以内的活产婴儿，得出发病率为1.37%。n澳大利亚1999-2003年通过出生缺陷等

3、级系统分析得出活产婴儿先心病发病率为1.09%。n研究发现，先心病发病率具有一定的规律，即出生时仅能发现40-50%的病例，出生1个月后可发现50-60%，1岁时可达90%左右。Etiologic Aspects&Prevention of CHDn遗传因素：遗传因素：n1.染色体畸变：CONGENITALHEARTDISEASES(CHD)INSELECTEDCHROMOSOMALABERRATIONSnPopulation studied Incidence Most commonn of CHD lesionsnGeneralpopulation1VSDPDAASDn4p-40VSDAS

4、DPDAn5p-25VSDPDAASDnCgroupanomalies5-50VSDPDAn13trisomy90VSDPDAn13q-50VSDn18trisomy99+VSDPDAPSn18q-50VSDn21trisomy50VSDAVcanalASDnXOTurner35COAASASDnXXXXT14PDAASDn2.单基因突变：单基因突变：n 常染色体显性遗传：常染色体显性遗传：Marfann Holt-Oramn 常染色体隐性遗传：常染色体隐性遗传：Ellis Van Greveldn Pompen 性染色体显性隐性遗传性染色体显性隐性遗传：n这类中多合并升主动脉扩张、动脉瘤、房

5、室间隔缺损、这类中多合并升主动脉扩张、动脉瘤、房室间隔缺损、单心房、心肌病单心房、心肌病n神经嵴发育异常神经嵴发育异常：n“畸形心脏是古老心脏”Taussign环境因素环境因素：teratogen(致畸因子)宫内感染：孕期前三个月宫内感染：孕期前三个月 理化因素：放射线、药物、农药等理化因素：放射线、药物、农药等 代谢紊乱：糖尿病代谢紊乱：糖尿病 宫内缺氧及不良嗜好：酗酒、吸毒宫内缺氧及不良嗜好：酗酒、吸毒n主要为多基因与环境因素主要为多基因与环境因素相互作用相互作用 多个致病基因相加多个致病基因相加 致病环境因素致病环境因素n预防的重点在妊娠预防的重点在妊娠期的前期的前三个月三个月CHD分类

6、分类nL to R:VSD ASD PDAnR to L:ToF TGAnNon-shunt:AS PS室间隔缺损室间隔缺损(VSD)Ventricular Septal DefectsnIn this case,the hole or defect is in the heart muscle forming a wall between the ventricles(the intraventricular septum).nThe heart can dilate,the muscle can become weak,and the pressures in the pulmonary

7、arteries can increase(pulmonary hypertension)due to the increase in blood flow.1.EPIDEMIOLOGYnItwasreportedinthatapproximately 20%of congenitalheart patients have a VSD as asolitary lesion.The incidence ofVSDinalllivebirthsisapproximately1.52.5per1,000.nRarely,theseholescanbeassociatedwithotherdefec

8、tsoftheheart.2.PATHOLOGY 2.PATHOLOGY3.PATHOPHYSIOLOGY 3.PATHOPHYSIOLOGY nOxygenatedbloodfromtheleftventricleshuntsviatheholeintotherightventricle.Thisvolumeloadcausesenlargementofbothventriclesandthepulmonaryartery,andexposestherightventricleandpulmonaryarteriestoabnormallyhighpressures.3.PATHOPHYSI

9、OLOGYnRV、PA、LA、LV的血流量增加的血流量增加nA、V、RA的血流量下降的血流量下降RVRA VPCLALV A 3.PATHOPHYSIOLOGY n1.Pulmonary Hypertension Eisenmenger SyndromeSmall defectModerate defectLarge defectslow pulmonary vascular resistancevariable pulmonary vascular resistancemarked elevation ofpulmonary vascular resistance3.PATHOPHYSIOL

10、OGYn与与缺缺损损口口面面积积(分分流流量量)及及肺肺血血管管对对高高流流量量、高冲击力的反应强弱而不同高冲击力的反应强弱而不同肺小动脉痉挛肺小动脉痉挛动力性动力性PAH-可逆可逆肺小动脉肌层、内膜增厚肺小动脉肌层、内膜增厚 (变性变性)阻力性阻力性PAH-不可逆不可逆 3.PATHOPHYSIOLOGY n2.Heart Failure:多见于多见于1Y和和14Yn3.SBE：多见于多见于1Y，30Y少有发生少有发生n4.Pneumonian5.自发闭合自发闭合4.MANIFESTATIONS(Clinical Features)n1.History:Small Defects(0.5cm

11、)症状轻症状轻Moderate-sized Defects(0.5-1.0cm)症状明显症状明显Large Defects(1.0cm)症状重症状重4.MANIFESTATIONSnInthefirst1to2weeksoflife,babieswithlargeventricularseptaldefectsmaydoverywell.Butasthepressureintherightsideoftheheartdecreases,bloodwillstarttoflowtothepathofleastresistance.Thiswillgraduallyleadtosymptoms(i

12、.e.,failuretothrive,feedingdifficulties,congestiveheartfailure.)andmustbetreated.4.MANIFESTATIONSnSmall ventricular septal defectsrarelycauseproblems.Aphysicianusuallydiscoverstheseholesbynoticingamurmuronaroutinephysicalexam.nMostoftheseholeswillcloseontheirown,particularlyiftheyareinthemuscularpor

13、tionoftheseptum.Eveniftheseholesdonotclose,theywillrarelycauseanyhealthproblems.4.MANIFESTATIONSnLarge ventricular septal defectscancauseproblems,ofteninthefirstfewmonthsoflife.4.MANIFESTATIONSnMedium or moderate ventricular septal defectsaremorechallengingtopredict.Sometimesbabiesbornwithmoderateve

14、ntricularseptaldefectswillhaveproblemswithcongestiveheartfailurelikebabieswithlargeventricularseptaldefects.Otherswillhavenoproblemsatallandjustneedtobewatched.4.MANIFESTATIONSn2.Physical Examination:望望心前区隆起心前区隆起 心尖搏动弥散心尖搏动弥散触触L3-4触及收缩期震颤触及收缩期震颤扣扣心浊音界向左扩大心浊音界向左扩大听听l1.L3-4闻及闻及3-46 SM,粗糙粗糙,向心向心尖尖 部及背部

15、广泛传导部及背部广泛传导l2.P亢强亢强l3.心尖部的心尖部的DM-二尖瓣相对狭窄二尖瓣相对狭窄5.SPECIFIC EXAMINATIONS nEKG5.SPECIFIC EXAMINATIONS5.SPECIFIC EXAMINATIONSnEcho嵴下型嵴下型嵴下型嵴下型VSDVSD5.SPECIFIC EXAMINATIONS隔瓣后型隔瓣后型隔瓣后型隔瓣后型VSDVSD5.SPECIFIC EXAMINATIONS干下型干下型干下型干下型VSDVSD5.SPECIFIC EXAMINATIONS肌部肌部肌部肌部VSDVSD5.SPECIFIC EXAMINATIONS肌部室间隔缺损彩色

16、血流图肌部室间隔缺损彩色血流图肌部室间隔缺损彩色血流图肌部室间隔缺损彩色血流图5.SPECIFIC EXAMINATIONS室间隔膜部瘤室间隔膜部瘤室间隔膜部瘤室间隔膜部瘤5.SPECIFIC EXAMINATIONS单心室单心室单心室单心室5.SPECIFIC EXAMINATIONS5.SPECIFIC EXAMINATIONSMembraneous VSD5.SPECIFIC EXAMINATIONS5.SPECIFIC EXAMINATIONSnThe purposes of the CC are primarily to:ndocumentthepresenceofthedefect

17、ordefects;evaluatethemagnitudeofshuntingacrossthedefect;estimatepulmonary vascular resistance;estimatetheworkloadofthetwoventricles;document or rule out thepresence of associated defects,andtoprovidethesurgeonwithaclearanatomicpictureofthelocationofthedefects.5.SPECIFIC EXAMINATIONSCatheter courses

18、from the inferior vena cava into the right atrium,then through a patent foramen ovale into the left atrium then left ventricle.5.SPECIFIC EXAMINATIONS5.SPECIFIC EXAMINATIONS5.SPECIFIC EXAMINATIONS5.SPECIFIC EXAMINATIONS6.TREATMENTn1、一般治疗和并发症的处理：一般治疗和并发症的处理：纠正一般情况呼吸道感染的处理肺动脉高压的处理SBE的治疗6.TREATMENTnMan

19、ytimesobservationistheonlytreatmentneeded,withregularcheckupswiththecardiologist.Thismaybeasinfrequentaseverytwotothreeyearsinolderchildrenwithsmallventricularseptaldefectstoweeklyinbabieswithlargeventricularseptaldefects.6.TREATMENTnOPERATIONBefore operationAfter operation6.TREATMENTnInterventional

20、TreatmentofVSDAmplatzerSeptalOccluder6.TREATMENT6.TREATMENT6.TREATMENT6.TREATMENT6.TREATMENT 房间隔缺损房间隔缺损(ASD)Atrial Septal DefectsnThis defect is characterized by incomplete closure between the two upper chambers of the heart.Blood can therefore flow through a hole(defect)in the wall(septum)between t

21、he upper chambers of the heart(atria)from one side to the other,causing some heart chambers to have to pump extra blood.nThe heart can dilate,the muscle can become weak,and the pressures in the pulmonary arteries can increase(pulmonary hypertension)due to the increase in blood flow.If the defect clo

22、ses on its own or with the aid of interventional therapy or surgery,these consequences can be avoided.1.EPIDEMIOLOGY1.EPIDEMIOLOGYnsASD occurred in 2030%of patient,the ratio of affected females to males is 2 1.nMost small ASDs will close spontaneously by age 3 years.2.2.2.2.PATHOLOGIC ANATOMYPATHOLO

23、GIC ANATOMY nForamenSecundum:MostMostncommontypeofASDn(60%).Defectislocatedatatnthefossaovalis(midnportionoftheatrialnseptum).2.2.PATHOLOGIC ANATOMY 2.2.PATHOLOGIC ANATOMYnSinusVenosus:DefectisisnhighinposteriorportionnoftheinteratrialseptumnandtheSVCusuallynoverridesthedefect.nSinusvenosusASDsarear

24、enassociatedwithpartialnanomalouspulmonarynvenousreturn.3.PHYSIOLOGYnRA、RV、PA、LAnLVn Although the size of the defects affects the flow of blood from the left to the right atrium,the defects usually large and shunt depends on the respective filling resistances in the right and left ventricles.3.PATHO

25、PHYSIOLOGYnRA、RV、PA、LA的血流量增加的血流量增加nA、V、LV的血流量下降的血流量下降RVRAVPCLALVA4.MANIFESTATIONSnInmostchildren,atrialseptaldefectscausenosymptoms.Averylargedefectmayallowsomuchbloodflowthroughittocausecongestiveheartfailuresymptomssuchasshortnessofbreath,easyfatigability,orpoorgrowth.4.MANIFESTATIONSnMostoftenana

26、trialseptaldefectisdiagnosedwhenaphysicianhearsaheartmurmurduringaphysicalexamination.4.MANIFESTATIONSn望望：心尖搏动弥散心尖搏动弥散 心前区隆起心前区隆起n触：（触：（）n扣：心浊音界扩大扣：心浊音界扩大n听：听：1.L2-3 2-3/6 SM柔和柔和 传导不明显传导不明显n2.P亢强亢强 固定分裂固定分裂n3.三尖瓣区三尖瓣区DMn 肺动脉瓣区收缩早期喷射音肺动脉瓣区收缩早期喷射音4.MANIFESTATIONSnThemurmurdoesntactuallycomefrombloodgo

27、ingacrossthehole,butratherfromthepulmonaryvalveareabecausetheheartisforcinganunusuallylargeamountofbloodthroughanormalsizedvalve.nThesecondheartsoundischaracteristicallysplitwhichisdifferentthanwhatisheardwhenlisteningtoanormalheart.5.SPECIAL EXAMINATION5.SPECIAL EXAMINATION胸部侧位片5.SPECIAL EXAMINATIO

28、N5.SPECIAL EXAMINATION5.SPECIAL EXAMINATION5.SPECIAL EXAMINATION 5.SPECIAL EXAMINATION继发孔型继发孔型继发孔型继发孔型ASDASD5.SPECIAL EXAMINATION原发孔型原发孔型原发孔型原发孔型ASDASD5.SPECIAL EXAMINATION上腔型上腔型上腔型上腔型ASDASD5.SPECIAL EXAMINATIONASDASD右心扩大右心扩大右心扩大右心扩大5.SPECIAL EXAMINATION房间隔分流的彩色血流图房间隔分流的彩色血流图5.SPECIAL EXAMINATION原发孔

29、房间隔缺损彩色血流图原发孔房间隔缺损彩色血流图原发孔房间隔缺损彩色血流图原发孔房间隔缺损彩色血流图5.SPECIAL EXAMINATION上腔型房间隔缺损彩色血流图上腔型房间隔缺损彩色血流图上腔型房间隔缺损彩色血流图上腔型房间隔缺损彩色血流图5.SPECIAL EXAMINATION多发房间隔缺损的彩色血流图多发房间隔缺损的彩色血流图多发房间隔缺损的彩色血流图多发房间隔缺损的彩色血流图5.SPECIAL EXAMINATION5.SPECIAL EXAMINATION5.SPECIAL EXAMINATION原发孔型原发孔型ASD5.SPECIAL EXAMINATION5.SPECIAL

30、EXAMINATIONASD5.SPECIAL EXAMINATION5.SPECIAL EXAMINATION6.TREATMENTnInsomechildrenanASDmaycloseonitsownwithouttreatment.Withasmallatrialseptaldefect,thismaybeashighas80percentinthefirst18monthsoflife.AnASDstillpresentby3yearsofagewillprobablynevercloseonitsown.6.TREATMENT nAmplatzerSeptalOccludernDe

31、pendingonthesizeandtheareaoftheseptuminvolved,manyatrialseptaldefectsmaybeclosedbyplacementofadevicecalledanAmplatzerSeptalOccluderduringacardiaccatheterization.ThisdeviceisinsertedthroughacatheterandcoverstheASDbyattachingtotheatrialseptum.6.TREATMENT nThebenefitsofbeingabletocloseanatrialseptaldef

32、ectwithanAmplatzerdeviceisthatitcanbeputinplacewithoutstoppingthepatientsheartorutilizingcardiopulmonarybypass,itdoesnthavethepsychologicaltraumarelatedtoopen-heartsurgeryanditdoesntcreateascarringacrossthechestthewayopen-heartsurgerydoes.6.TREATMENT 6.TREATMENT6.TREATMENT6.TREATMENT6.TREATMENTHow t

33、o How to measure measure diameter of diameter of ASDASDballoonballoon6.TREATMENT6.TREATMENT6.TREATMENT6.TREATMENT6.TREATMENTLARASVCIVCLARARVLV6.TREATMENTnOpen-HeartSurgerynAnatrialseptaldefectismostcommonlyclosedbyopen-heartsurgery.Thesurgeonmaybeabletodirectlyclosetheholewithsuturesor,dependingonth

34、esizeandshapeofthehole,mayneedtocloseitwithapatch.6.TREATMENTnAtrialseptaldefectclosureresultsnSurgicalclosureofatrialseptaldefectsiscomplicationfreeinover99%ofcases.AlthoughtheAmplatzerdevicehasonlybeenutilizedforseveralyears,itssuccessratealsoappearstobeveryhigh.AfterASDclosureinchildhood,theheartsizereturnstonormaloveraperiodoffourtosixmonths.n复习题复习题n简述室间隔缺损的血流动力学改变简述室间隔缺损的血流动力学改变n房间隔缺损的诊断要点房间隔缺损的诊断要点n室间隔缺损的诊断要点室间隔缺损的诊断要点n如何预防先天性心脏病如何预防先天性心脏病