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先天性心脏病CongenitalHeartDisease(CHD),Dept.Pediatricof2ndXiangyaHospitalofCSU,WangCheng,中南大学儿科学教研室,第十三章,概述Overview,CHDisdefinedasanabnormalityincirculatorystructureorfunctionthatispresentatbirth,evenifitisdiscoveredmuchlater,CHDispresentin0.687%inChina,先天性心脏病是胎儿时期心脏血管发育异常而导致的先天性畸形,出生后1年内先天性心血管发育畸形出现率占活产婴儿的6.87,Bytheprogressionofallkindsofinspectiontechniquesandinterventionaltherapy,theprognosisofCHDhastakenonanewlook.,概述Overview,由于各种心血管检查技术（特别是彩色多普勒超声心动图）的应用，深低温麻醉和体外循环下心内直视手术的发展，先心病介入性治疗进展，临床上对先心病的诊断和治疗发生了很大变化，预后大为改观。,目的要求ObjectiveandRequest,掌握室间隔缺损、房间隔缺损、动脉导管未闭、法洛四联症的血流动力学、临床表现及常见并发症TomasterthehemodynamicsandclinicalsituationandthediagnosisofcommoncomplicationsinVSD,ASD,PDAandTOF,熟悉先天性心脏病的病因、分类、预防及治疗原则FamiliarwiththeetiologyandclassificationofCHD,病因Etiology,遗传因素(内在因素)与基因突变、染色体畸变有关Geneticfactor(internalfactor)CHDresultfromgenemutationorchromosomeaberration,环境因素(外界因素)与宫内病毒感染有关Environmentalfactor(externalfactor)CHDismainlycorrelationtotheintrauterineinfection,预防Prevention,加强孕妇保健Thehealthprotectionofpregnantwomanshouldbeenhanced,避免接触药物、辐射等高危因素Highriskfactorsshouldbeavoided,妊娠早期适量补充叶酸SuitdosageFolicAcidshouldbefilledupinearlypregnancystage,分类Classification,左向右分流型(潜在青紫型)left-to-rightshunts(noncyanosis),右向左分流型(青紫型)right-to-leftshunts(cyanosis),无分流型(无青紫型)nonshunts(noncyanosis),PS,VSD,TOF,临床常见的先心病CommonCHDinClinic,室间隔缺损Ventricularseptaldefect(VSD),房间隔缺损Atrialseptaldefect(ASD),动脉导管未闭Patentductusarteriosus(PDA),法洛四联症TetralogyofFallot(TOF),室间隔缺损VentricularSeptalDefect（VSD）,室间隔缺损血流动力学示意图HemodynamicsFigureofVSD,PV,SVC,RA,PA,IVC,RV,PV,LA,AO,LV,肺静脉,上腔静脉,右心房,肺动脉,下腔静脉,右心室,肺静脉,左心房,主动脉,左心室,VSDmurmur,室间隔缺损血流动力学变化HemodynamicsofVSD,小室缺可无血流动力学变化SmallVSDnohemodynamicschanges,大室缺大量左向右分流LargeVSDLargeleft-to-rightshunts,动力型肺动脉高压DynamicPH,梗阻型肺动脉高压ObstructedPH,艾森门格综合征EisenmengerSyndrome,肺动脉高压PulmonaryHypertension(PH),VSD临床表现ClinicalfindingsofVSD,症状Symptoms,反复呼吸道感染，生长发育落后、乏力、气短、声嘶frequentrespiratoryinfections,growslowly,verypoorweight,dyspnea,exerciseintolerance,fatigue,congestiveheartfailure,体征Signs,LSB3-4可闻3-4/6级粗糙全收缩期吹风样杂音，向周围广泛传导，伴震颤Agrade-/,medium-to-highpitched,harshpansystolicmurmurattheleftsternalborder(LSB)inthe3rdand4thintercostalspaces,肺动脉瓣第二音亢进ThepulmonarycomponentofS2isaccentuationinintensity,合并主动脉瓣关闭不全时可闻舒张期杂音AdiastolicmurmurindicateVSDcombinewithaorticregurgitation,室间隔缺损并发症ComplicationofVSD,支气管肺炎Bronchopneumonia充血性心力衰竭Congestiveheartfailure肺水肿Pulmonaryedema感染性心内膜炎Infectiveendocarditis,房间隔缺损AtrialSeptalDefect（ASD）,PV,LA,PA,LV,PV,SVC,RA,AO,IVC,RV,肺静脉,肺静脉,上腔静脉,右心房,主动脉,下腔静脉,右心室,左心房,肺动脉,左心室,房间隔缺损血流动力学示意图HemodynamicsFigureofASD,ASDmurmur,上、下腔静脉血肺静脉右心房（扩大）左心房右心室（增大）左心室（血量减少）肺血流量明显增加（肺充血）肺小动脉痉挛、增厚体循环供血不足右向左分流（消瘦、乏力、心悸、气短等）艾森门格综合征(少数病人晚期）,ASD,房间隔缺损血流动力学变化HemodynamicsofASD,症状Symptoms,同室缺相似SymptomsofASDaresimilarwiththatofVSD,体征Signs,LSB2-3可闻1-3/6级柔和收缩期喷射音，无震颤Agrade-/ejectionSMisheardbestattheLSBinthe2ndintercostalspace，nothrill,P2固定分裂S2atthepulmonaryareaiswidelysplitandoftenfixed,肺动脉瓣第二音亢进ThepulmonarycomponentofS2isaccentuationinintensity,三尖瓣区可闻舒张期杂音Amid-diastolicmurmurcanoftenbeheardintricuspidarea,ASD临床表现ClinicalfindingsofASD,房间隔缺损并发症ComplicationofASD,支气管肺炎Bronchopneumonia充血性心力衰竭Congestiveheartfailure感染性心内膜炎Infectiveendocarditis,动脉导管未闭Patentductusarteriosus(PDA）,PV,SVC,RA,PA,IVC,RV,PDA,PV,LA,AO,LV,肺静脉,肺静脉,上腔静脉,下腔静脉,右心房,主动脉,肺动脉,右心室,左心房,左心室,动脉导管未闭血流动力学示意图HemodynamicsFigureofPDA,动脉导管,PDAmurmur,右心室血流体循环舒张压肺动脉主动脉供血减少脉压增宽肺血流量肺动脉高压艾森门格综合征左房、左室扩大（差异性紫绀）（右心室肥大）,PDA,动脉导管未闭血流动力学变化HemodynamicsofPDA,症状Symptoms,与VSD及ASD相同SymptomsofPDAaresimilarwiththatofVSDandASD,体征Signs,LSB2闻及粗糙、响亮的连续性机器样杂音，占据整个收缩期及舒张期Acharacteristicthrillandcontinuousandrough“machinery”murmurwithalatesystolicaccentuation,心尖区可闻及舒张中期隆隆样杂音Adiastolicflowmurmurisoftenheardattheapex,周围血管征Peripheralvascularsigh,下半身青紫(差异性紫绀)和杵状趾Differentialcyanosisandclubbing,PDA临床表现ClinicalfindingsofPDA,PDA堵闭前心音图,S1,S2,PDA堵闭后心音图,S1,S2,动脉导管未闭并发症ComplicationofPDA,支气管肺炎Bronchopneumonia感染性心内膜炎Infectiveendocarditis充血性心力衰竭Congestiveheartfailure,左向右分流先心病共同临床特点Commonfeaturesofleft-to-rightshuntingCHD,一般情况下无青紫Ingeneraltherearenoncyanosis心前区有粗糙的收缩期杂音Aroughsystolicmurmurisheardinprecordium肺循环血量多，易患肺炎Pulmonaryflowincreaseandeasytosufferfrombronchopneumonia体循环血量少，影响生长发育Systemicflowdecreaseandthepatientgrowslowly,法洛四联症TetralogyofFallot（TOF）,PV,SVC,RA,PA,IVC,RV,PV,LA,AO,LV,肺静脉,肺静脉,上腔静脉,右心房,肺动脉,下腔静脉,右心室,左心房,主动脉,左心室,法洛四联症血流动力学示意图HemodynamicsFigureofTOF,TOFmurmur,法洛四联症四种解剖畸形ThefourmalformationsofTOF,肺动脉狭窄Obstructiontorightventricularoutflow室间隔缺损Ventricularseptaldefect主动脉骑跨Overridingoftheaorta右心室肥厚Rightventricularhypertrophy,右心室（肥厚）左心室肺动脉狭窄主动脉（血流量、扩张）肺血流量减少（肺野清晰）混合血进入循环（青紫、发育落后、乏力血氧合不足（杵状指趾等）蹲踞、阵发性昏厥）,分流,分流,法洛四联症血流动力学变化HemodynamicsofTOF,青紫：本病最突出的症状Cyanosisisthemainsymptoms,气促和缺氧发作Dyspneaandhypoxemicspells,蹲踞症状Squattingposture,心脏体征LSB2-4闻及2-4/6级收缩期喷射性杂音Thereisagrade-/,rough,ejection-typeSMthatismaximalattheLSBinthe2ndto3rdintercostalspaceandthatradiateswelltotheback,杵状指趾Clubbingoftheterminaldigits,TOF临床表现ClinicalfindingsofTOF,杵状指Clubbingoffingers,杵状指趾Clubbingoffingersandtoes,脑血栓Cerebralembolism脑脓肿Cerebralabscess感染性心内膜炎Infectiveendocarditis,法洛四联症并发症ComplicationofTOF,XRayofTOF,X线检查右室大、心尖上翘呈靴形，肺动脉段凹陷，肺野清晰,先天性心脏病的诊断DiagnosisofCHD,病史History体查Physicalexamination心电图Electrocardiogram心脏X线检查ChestX-ray超声心动图Echocardiogram心导管检查和心血管造影Cardiaccatheterizationandangiocardiography,临床上有诊断意义的心脏杂音Clinicalsignificanceheartmurmur,全收缩期杂音Pansystolicmurmur收缩晚期杂音Latesystolicmurmur舒张期杂音Diastolicmurmur连续性杂音Continuousmurmur强度3/6级的杂音Grade3/6murmur,先天性心脏病的治疗TreatmentofCHD,内科治疗Medicalmanagement,治疗心衰，良好护理Anticongestiveheartfailuremeasures,goodnursing,外科治疗Operationtreatment,开胸心脏修补手术Repairthed