CongenitalHeartDisease(CHD)ShengjingHospitalPediatricsYuXuexinPediatrics,ShengjingHospitalIntroductionIntroduction•CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater.•Incidence:6.9‰inaliveneonatal.•150,000neonatalsufferfromCHDinChinaperyear.•Newtreatments:catheterization、developmentofoperation,etc.Pediatrics,ShengjingHospitalObjectandRequestObjectandRequest•FamiliarwiththeetiologyandclassificationofCHD.•Masterthehemodynamics、clinicalmenifestationanddiagnosisofcommoncomplicationsinVSD,ASD,PDAandTOF.Pediatrics,ShengjingHospitalEtiologyEtiology•Internalfactors:genemutationorchromosomeaberration.•Externalfactors:intraureteralinfection、ray、drug、metabolicdiseases、intraureteralhypoxia.Pediatrics,ShengjingHospitalClassificationClassificationleft-to-rightshunts•Cyanosismaybe•VSD、PDA、ASDright-to-leftshuntsnon-shunts•Cyanosis•TOF、dislocationofmainartery•Pulmonaryarterystenosis、aorticstenosisBasedonshuntbetweenrightandleftheartPediatrics,ShengjingHospitalPatentductusarteriosusPDAPatentductusarteriosusPDACommonCHDinClinicCommonCHDinClinicAtrialseptaldefectASDAtrialseptaldefectASDVentricularseptaldefectVSDVentricularseptaldefectVSDTetralogyofFallotTOFTetralogyofFallotTOF11223344Pediatrics,ShengjingHospitalVentricularSeptalDefect(VSD)1、membranedefect85%2、musculardefect3、funneldefect10%20-50%VSDcancloseupwithouttreatment.1、minordefect2、mediadefect3、majordefectAnatomyMostcommon,30%inCHD。Pediatrics,ShengjingHospitalPathobiologyPathobiologyRVblood↑,pulmonaryhypertension,persistentcyanosis(Eisenmengersyndrome)LVblood↓,bodycirculation↓Pediatrics,ShengjingHospitalHemodynamicsHemodynamicsBeforepulmonaryhypertensionRARV(blood↑)Pulmonaryartery(dilation)Pulmonarycirclation(congestion)RV(dilation)LA(hypertrophy)LV(hypertrophy)(射血量减少)bodycirculationBloodvolume↓shuntPediatrics,ShengjingHospitalHemodynamicsHemodynamicsBodycirculation(mixedblood)RALAPulmonaryArterydilationRV(Dilation)AfterpulmonaryhypertensionLVDynamicPulmonaryhypertesionObstructivepulmonaryhypertesionshuntPediatrics,ShengjingHospitalClinicalManifestationClinicalManifestation•症状:分流量大时:生长迟缓、体重不增、消瘦、喂养困难、活动后乏力、气短、多汗、反复呼吸道感染、心衰。声音嘶哑(肺动脉压迫喉返神经)。•体征:胸骨左缘3、4肋间Ⅲ-Ⅳ粗糙的全收缩期杂音,向四周传导,伴有震颤。肺动脉第二音亢进。二尖瓣相对狭窄的较柔和舒张中期杂音Pediatrics,ShengjingHospitalExaminationExamination•X线:左、右心室增大,以左室增大为主,主动脉弓影较小,肺动脉段突出,肺野充血。艾森曼格综合征:肺动脉主支增粗,肺外周血管影很少,宛如枯萎的枯枝。•心电图•USPediatrics,ShengjingHospitalComplicationsandtreatmentComplicationsandtreatment•合并症支气管肺炎、心衰、肺水肿、亚急性细菌性心内膜炎•治疗小型缺损:不一定手术治疗。中型缺损:5-6岁做手术。大型缺损并反复心衰者:可在6月-2岁内做手术。介入治疗Pediatrics,ShengjingHospitalAtrialAtrialSeptalDefectASDAtrialAtrialSeptalDefectASD•5-10%•病理解剖:1.原发孔型:约占15%,缺损位于心内膜垫与房间隔交界处。2.继发孔型:中央型,最常见,约占75%,缺损位于房间隔中心卵圆窝。3.静脉窦型:约占5%,分为上腔型和下腔型。4.冠状静脉窦型:约占2%,缺损位于冠状静脉窦上端与左心房间。Pediatrics,ShengjingHospitalAtrialAtrialSeptalDefectASDAtrialAtrialSeptalDefectASDHemodynamicsHemodynamicsPediatrics,ShengjingHospitalHemodynamicChangeHe...