Chapter13循环系统疾病 Circulationsystemdisease 教学目的初步熟悉 了解先天性心脏病掌握先天性心脏病的诊断和治疗教学重点详细介绍先天性心脏病的临床表现和诊断方法教学难点先天性心脏病血液动力学改变与临床表现的关系 Contents 小儿心血管系统解剖生理特点及检查方法小儿先天性心脏病概述房间隔缺损室间隔缺损动脉导管未闭法洛四联症 第一节小儿心血管系统解剖生理特点及检查方法 一心脏的胚胎发育Cardiacdevelopmentduringfetalperiod Theprimitivehearttubeformsby3WCardiacloopingby22 24daysBendventrallyandtowardtherightCardiacseptation 胚胎房室分隔及房室间隔发育 室间隔形成心室间隔组成三个来源 肌隔 心内膜垫向下生长与肌隔相合 动脉总干及心球分化成主动脉与肺动脉时的中隔向下延伸部分 室间隔膜部 Aorticarchdevelopment RightandlefttruncoconalridgesTheaorticandpulmonaryoutflowtractsarefullyseparatedThecoronarysinusThesemilunarvalves 主肺动脉由总干呈螺旋形分隔 大动脉位置变移系列示意图 TOF TGA DORV Taussig Bing 房室管 心房 及心室的分隔 室间隔膜部的形成及室间隔的封闭 第2周原始心脏形成第4周心脏起循环作用第8周四腔心脏形成 二胎儿新生儿循环转换 Theplacenta gasexchange metabolicexchangeThelung constrictedThreecardiovascularstructure DuctusvenosusForamenovaleDuctusarteriosus TheFetalCirculation 胎儿血循环特点 营养和气体代谢通过脐血管和胎盘与母体进行弥漫式交换绝大部分为混合血供应心脏 脑 肝及上肢血氧含量远较下半身高肺处于压缩状态无气体交换卵圆孔 动脉导管和静脉导管开放右心室优势 TheFetalCirculation 胎儿血液循环途径 通过动脉导管 通过卵圆孔 血氧含量较高 血氧含量较低 通过静脉导管 50 1 3 80 PDA CirculationChangesafterbirth TheumbilicalcordclampedbreathingcommencesPulmonarycirculationProvidinggasexchangeTheforamenovaleandductusarteriosus functionallyclosed 胎儿与出生后血液循环比较 返回 正常血液循环途径 Commonsymtoms CongestiveheartfailureFeedingdifficulties ExerciseintoleranceRespiratorydistressPoorgrowthCracked coughSquattingSyncopeCyanosis 三儿童心血管疾病体格检查方法 Physicalexamination CommonlymanifestationsEvaluationforBWandBHRespiratorydistressCyanosisClubbingdigitsHepatomegalyDistendedneckveinsSignofliver jugularveinPeripheraledema CardiovascularExamination Inspection ProminenceoftheprecordiumCardiomegalyPalpation heave thrillPercussion sizeandpositionoftheheartAuscultation HR rhythm 1 Normalheartsounds S1 S2 S3 S4 2 Abnormalheartsounds P2fixedsplitting 3 Murmurs 小儿听诊常用听诊区二尖瓣听诊区肺动脉瓣听诊区主动脉瓣听诊区三尖瓣听诊区 1 Locationandradiation 2 Timing Systolic diastolicandcontinuous 3 Quality Harsh musical orrough high mediumorlowinpitch 4 Intensity grade 5 Variationwithposition Murmur 周围血管征 四肢脉搏及血压主动脉缩窄 股动脉搏动减弱或消失下肢血压低于上肢动脉导管未闭或主动脉关闭不全 脉压增宽毛细血管搏动股动脉枪击音 四心脏的特殊检查 1 Chestx ray 2 Electrocardiogram 3 Echocardiogram 4 Cardiaccatheterization 5 Angiocardiography 6 Radionuclideangiocardiography 7 Magneticresonanceimaging 8 Computedtomography ChestRadiograph characterofthepulmonaryvasculaturePositionofthecardiacapexcardiacsize cardiothoracicratio cardiacconfiguration heartshapePositionoftheabdominalviscera Normalcardiothoracicimaginglandmarks PAview Anteriorradiographicviewofcardiacanatomy X ray ThesequenceofECG rate rhythm Pwave PRinterval QRScomplex axis amplitude andduration QTinterval STsegment andTwave AssessatrioventricularhypertrophyDiagnosisofdysrhythmias Electrocardiogram ECG NormalECG Apowerfulnoninvasivemethod M mode twoandthreedimensional colorandDopplerEvaluatecardiacstructureEstimateintracardiacpressureandgradientacrossstenoticvalvesandvesselsQuantitatecardiaccontractilefunctionDeterminethedirectionofflowacrossadefectExaminetheintegrityofthecoronaryarteriesEvaluateendocarditis pericardiacfluid cardiactumors orchamberthrombi Echocardiography M modeECHO Long axisviewforLV 2 DECHO Four chamberviewatapex 2 DECHO Short axisviewatthegreatartery 2 DcolorECHO four chamberview 2 DcolorDopplerECHO 3 DReconstruction SVC IVC RV CardiacCatheterization EstimateCardiacoutputaccordingtoOxygencontentandsaturation pulmonaryandsystemicbloodflow DeterminethepressuresinallchambersandvesselsCalculatethepulmonaryandsystemicvascularresistanceInterventionalcatheterization therapeuticprocedures Angiocardiography 选择性造影Selectiveangiocardiography数字减影造影 DSA 诊断复杂先心病的主要手段常用成角造影长轴斜位 左前斜60 75 向头成角20 30 肝锁位 左前斜40 45 向头成角40 坐观位 正位向头成角40 心血管造影 心血管造影 Radionuclideangiocardiography 99m锝iv释放 射线初次循环心脏造影 平衡心脏血池造影检测左向右分流评估心功能 Magneticresonanceimaging NoninvasivetoolFollow upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethoraxPaulC Lauterbur UnitedStates andSirPeterMansfield Britain winedthe2003NobelPrizefordiscoveriesinmagneticresonanceimaging SE AO PA AO LV LA RV CineMRI Cine MRI MRA 3 DMRI MRIisinvaluableinthelongtermfollow upofcoarctationoftheaortaafterangioplasty surgicalprocedure Computedtomography Follow upEvaluatetheheartandthegreatarteriesProvidetheimagingofvascularstructuresofthethorax 第二节小儿先天性心脏病概述 概念 CHD是胎儿期心脏及大血管发育异常而致的心血管畸形 是小儿最常见的心脏病发病率 6 8 上海6 87 我国每年出生15万CHD诊治研究取得很大进展 预后大为改观 病因 内因 与遗传有关 染色体异常或基因突变 外因 1 早期宫内感染 风疹 流感 腮腺炎 柯萨奇病毒等 2 孕母接触大量放射线服用药物史 抗癌药 抗癫痫药 3 代谢性疾病 糖尿病 高钙血症 4 宫内慢性缺氧 5 妊娠早期酗酒 吸食毒品环境因素 遗传因素 Categoriesofcongenitalheartdisease Theleft to rightshuntlesions ASDVSDPDA AcyanoticearlyPulmonarybloodincreasedSystemicblooddecreasedPulmonaryhypertensionEisenmengersyndrome cyanoticlatelyduetoright to leftshunts Theright to leftshuntlesions TetralogyofFallot TOF transpositionofthegreatarteries TGA CyanoticearlyCyanoticlesionswithincreasedpulmonarybloodflowCyanoticlesionswithdecreasedpulmonarybloodflowThesystemiccirculationishypoxemia No shuntlesions Pulmonarystenosis PS Coarctationoftheaorta Coa AcyanoticHypertrophyresultinginincreasedpressureload Va