1、Echocardiography of Congenital Heart DiseaseThe 1st clinical college of GZMU The ductus arteriosus usually constricts shortly after birth(within 24-48hours)turns into the ligamentum arteriosum,once the left-sided pressures exceed the right-sided pressures.If this communication persists,it is called
2、patent ductus arteriosus.Very common congenital lesion of the heart,amounting to 10-15%of congenital heart disease.是常见先天心之一,占先天心10-15%。可单独存在，亦可合并其它畸形存在。Ductus arteriosus located between the isthmus of aortic arch and the root of left pulmonary artery.Diameter of the duct is 6-10mm.Types:tube-infundi
3、buliform-window-动脉导管位于主动脉弓峡部与左肺动脉根动脉导管位于主动脉弓峡部与左肺动脉根部之间。部之间。导管直径差别较大，多数导管直径差别较大，多数1.0cm左左右，常以右，常以0.6cm-1.0cm多见。多见。分型：分型：根据形态学分管型、漏斗型、窗型。根据形态学分管型、漏斗型、窗型。Shunt flow between Great artery level:Aortapulmonary artery,continuously shunt in diastolic phase and systolic phase.Too much shunt causes volume ov
4、erloading in pulmonary circulation and left ventricle.Eisenmenger Syndrome occurs in serious cases in their advanced phase,bidirectional shunt or right-to-left shunt.产生大动脉水平左产生大动脉水平左右分流，为收缩、舒右分流，为收缩、舒张期连续性分流。张期连续性分流。分流量大引起肺循环及左心容量负荷过重分流量大引起肺循环及左心容量负荷过重。严重病例晚期出现肺高压（艾森曼格综合严重病例晚期出现肺高压（艾森曼格综合征），出现双向分流或右
5、向左分流。征），出现双向分流或右向左分流。The best echocardiographic views to visualize the PDA are:short axis view of the heart base,to show long axis view of pulmonary artery。suprasternal long axis view of the aortic arch.重点探查心底短轴或肺动脉长轴、胸骨上重点探查心底短轴或肺动脉长轴、胸骨上窝主动脉弓长轴观，寻找未闭动脉导管。窝主动脉弓长轴观，寻找未闭动脉导管。1、Direct signs：An opening
6、 duct can be visualized between:the descending aortathe bifurcation of pulmonary trunk the starting part of left pulmonary arterythe aortic archororand 1、直接征象直接征象：在肺动脉分叉或左肺动：在肺动脉分叉或左肺动脉近端与降主动脉之间显示有管道相通。脉近端与降主动脉之间显示有管道相通。根据形态分型，测量直径、长度。根据形态分型，测量直径、长度。（long axis view of pulmonary artery）LARARVMPALPARP
7、A久9（long axis view of the aortic arch）ARCHAAODAOLPAMPAPDA（long axis view of the aortic arch）2、indirect signs：Representation of volume overloading of left ventricle Dilated aorta and pulmonary artery,with enforced pulsating.M Mode:Pulmoary Valve in Pulmonarry Hypertension :“a”wave vanish，shaped“V”or“
8、W”.2、间接征象间接征象：可有左心容量负荷过重表现。：可有左心容量负荷过重表现。3、主动脉、肺动脉扩张，搏动增强。、主动脉、肺动脉扩张，搏动增强。合并肺动脉高压时，合并肺动脉高压时，显示肺动脉瓣曲线显示肺动脉瓣曲线a波消失，伴提前关闭呈波消失，伴提前关闭呈“V”或或“W”型。型。1、Color Doppler Flow Image：In the long axis view of pulmonary artery,red shunt flow along the external wall of pulmonary artery,while blue flow along internal
9、wall.（short axis view of the heart base）（Suprasternal long axis view of the aortic arch）9 1、彩色多普勒彩色多普勒：肺动脉长轴切面可见经导管进入肺动脉长轴切面可见经导管进入主肺动脉外侧壁、以红色为主彩流束。主肺动脉外侧壁、以红色为主彩流束。主肺动脉内侧可见蓝色血流束。主肺动脉内侧可见蓝色血流束。俄互=；俄互=；2、Pulsed doppler wave/continuous doppler wave：continuously,upward,high-speed,turbulent spectrum,sam
10、pling in the orifice of the dutus arteriosus.Peak velocity 4m/s。肺动脉长轴切面，取样容积置导管口处获肺动脉长轴切面，取样容积置导管口处获收缩、舒张期连续性正向高速湍流频谱。收缩、舒张期连续性正向高速湍流频谱。To estimate Pulmonary Artery Systolic Pressure(PASP)by spectrum doppler:（1）cross-ventricular septum formula through PDA shunt：P4V，P=LVSP-RVSP=AOSP PASP BASP-PASP;PA
11、SP=BASP-P=BASP-4V（V：peak velocity of PDA orifice,directly measured by CW）.RVRALALV（2）cross-tricuspid formula through tricuspid regurgitation ：P=RVSP-RASP=PASP RASP；P4VP4V，PASP=P+RASP=4V 4V+RASP.（V：peak velocity of tricuspid regurgitation,directly measured by CW）.RARVLALV（1）室间隔跨隔压差法：）室间隔跨隔压差法：P=LVSP-
12、RVSP=BASP-PASP;由于由于P=4V（V为为PDA处流速，频谱直接测量）处流速，频谱直接测量）因此因此 PASP=BASP 4V.（2）三尖瓣跨瓣压差法：）三尖瓣跨瓣压差法：P=RVSP-RASP=PASP-RASP 同理同理P=4V（V为三尖瓣返流流速，频谱直接测量）为三尖瓣返流流速，频谱直接测量）因此因此PASP=4V+RASP.1、B mode:Ductus arteriosus persists open 2、Doppler:left right shunt between aorta and pulmonary artery。3、With or without left v
13、entricular volume overloading。1、切面超声显示未闭动脉导管。、切面超声显示未闭动脉导管。2、多普勒超声显示上述导管内有分流信、多普勒超声显示上述导管内有分流信号。号。3、伴或不伴左心容量负荷过重。、伴或不伴左心容量负荷过重。1、Differ from disorder causing continuous cacophony:（1）aorta-pulmonary artery septum defect （2）VSD complicating AR.（3）Rupture of aortic sinus aneurysm.（4）Coronary-pulmonary
14、fistula.2、Different from disorder causing left ventricular volume overloading 1、与引起连续性杂音的疾病鉴别。、与引起连续性杂音的疾病鉴别。（1）主主-肺动脉间隔缺损肺动脉间隔缺损.（罕见）（罕见）（2）室间隔缺损并主动脉瓣关闭不全室间隔缺损并主动脉瓣关闭不全.（3）主动脉窦瘤破裂主动脉窦瘤破裂.（4）冠状动脉冠状动脉-肺动脉瘘肺动脉瘘.2、与引起左心容量负荷过重的疾病鉴别、与引起左心容量负荷过重的疾病鉴别。1.TTE:Affirmative diagnosis can be made.2.Help to deter
15、mine operation strategy.3.Evaluate the operation effect.目前应用经胸超声已能对目前应用经胸超声已能对多数单纯多数单纯PDA在术前获得诊断而直在术前获得诊断而直接手术，为术式的选择提供重要依接手术，为术式的选择提供重要依据，并用于评价手术效果。据，并用于评价手术效果。The endocardial cushion defect(ECD)is also called ostium primum atrial septum defect,artrioventricular canal malformation,artrioventricular
16、 septum defect(AVSD).These defects are subdivided into partial and complete forms.是指房室瓣水平上下的间隔组织发育不全，同时伴不同程度房室瓣发育异常的复合性先天畸形。病解分型：病解分型：1、部分型（不完全性）部分型（不完全性）2、完全型（完全完全型（完全性）性）.This condition results from primum atrial septum defect,and/or abnormal tricuspid valve,and/or cleft mitral valve.Left atrium t
17、o right atrium shuntMitral regurgitation（1）单纯原发孔房缺单纯原发孔房缺产生房水平左产生房水平左右分流。右分流。右心容量负荷过重。右心容量负荷过重。（2）原发孔房缺原发孔房缺+二尖瓣裂或二、三二尖瓣裂或二、三尖瓣裂尖瓣裂产生房水平左产生房水平左右分流及房右分流及房室瓣返流。室瓣返流。左、右心容量负荷过重。左、右心容量负荷过重。This defect has a single,undivided,free-floating leaflet stretching across both ventricles.This condition results f
18、rom a membranous ventricular septal defect,primum atrial septal defect,abnormal tricuspid valve,and cleft mitral valve.Atrioventricular valve regurgitation4 chambers communication PASP rise,even Heart Function Failure occurl具有具有原发孔房缺、室间隔膜部缺损原发孔房缺、室间隔膜部缺损，产生房、，产生房、室水平分流；室水平分流；l二、三尖瓣形成共同房室瓣（伴瓣裂），二、三尖瓣
19、形成共同房室瓣（伴瓣裂），产生产生房室瓣返流房室瓣返流。l四心腔血流互通四心腔血流互通 l早期出现肺动脉高压、心衰。早期出现肺动脉高压、心衰。俄互=；俄互=；chordal separately attach to the IVSA型：二、三尖瓣分开，腱索连于室间隔顶型：二、三尖瓣分开，腱索连于室间隔顶端。端。B型：二、三尖瓣分开，腱索附于室间隔右型：二、三尖瓣分开，腱索附于室间隔右室侧。室侧。C型：二、三尖瓣完全融为型：二、三尖瓣完全融为 一体，无腱索一体，无腱索相连，仅见膜样回声漂浮于房室瓣口。相连，仅见膜样回声漂浮于房室瓣口。Echocardiographically the ideal
20、 views are:the long-axis,short-axis-to search for abnormalities in the atrioventricular valves,such as presence of cleft.and four-chamber views-to search for chordal attachment,overriding,or straddling of the valves.The crux of the heart is carefully analyzed by sweeping the transducer anterior to p
21、osterior to record the outlet and inlet portions and the membranous septum.重点观察各四腔切面、二尖瓣口短轴切面、左重点观察各四腔切面、二尖瓣口短轴切面、左室长轴切面。室长轴切面。观察房、室间隔缺损。区别房室瓣结观察房、室间隔缺损。区别房室瓣结构类型及瓣叶附着点。构类型及瓣叶附着点。1.Defect of inferior atrial septum:primum atrial septal defect discontinuity of the anterior leaflet of mitral valve with
22、 the posterior wall of aorta 2D：Defect2.Mitral valve appears as invert“V”or“hanging bridge”when they open.3.Stenosis of left ventricular outlet4.Volume overloading of left/right ventricular may probably present.1）四腔心显示房间隔下部回声中断；）四腔心显示房间隔下部回声中断；2）短轴切面显示二尖瓣前叶瓣裂；）短轴切面显示二尖瓣前叶瓣裂；3）左室长轴显示二尖瓣前叶前移，左室流）左室长轴显
23、示二尖瓣前叶前移，左室流出道狭窄。出道狭窄。4）可有左、右室容量负荷过重表现。）可有左、右室容量负荷过重表现。1、Shunt：Red flow from LARA can be visualized in inferior atrial septum.2.Mitral valve regurgitation:Blue blood flow leak from LV through Mitral cleft into LA.（1）心房下部见左房）心房下部见左房右房红色为主穿隔右房红色为主穿隔分流束。分流束。（2）二尖瓣裂处见蓝色为主的多彩血流进）二尖瓣裂处见蓝色为主的多彩血流进入左房。入左房。1
24、.Including the sonogram as partial ECD2.Membranous ventricular septum defect 3.Mitral and tricusip valve is individed,located in the same level1.Including partial ECD2.Membranous VSD3.Common MV and TV （1）除有部分型表现外；）除有部分型表现外；（2）尚有室间隔膜部缺损；）尚有室间隔膜部缺损；心腔中部的心腔中部的“十十”字交叉结构消失；字交叉结构消失；二、三尖瓣形成共瓣，二、三尖瓣形成共瓣，二、三
25、尖瓣环处于等高位置。二、三尖瓣环处于等高位置。According to Chordal attachments,:A:Anterior mitral valve and tricuspid valve apart from each other;chordal separately attach to the IVSB:The chordal attachments from the medial portion of the cleft matral leaflet related to the papillary muscle on the right side of the septal
26、 defect.C:Free-floating common atrioventricular leafletA型型：二、三尖瓣分开，腱索连于室间隔顶：二、三尖瓣分开，腱索连于室间隔顶端。端。B型型：二、三尖瓣分开，腱索附于室间隔右：二、三尖瓣分开，腱索附于室间隔右室侧。室侧。C型型：二、三尖瓣完全融为一体，无腱索相：二、三尖瓣完全融为一体，无腱索相连，仅见膜样回声漂浮于房室瓣口。连，仅见膜样回声漂浮于房室瓣口。SYSTOLIC PHASE DIASTOLIC PHASE Diastolic phase When pulmonary hypertension occurs,bidiretion
27、al shunt between LV and RV（1）同不完全性）同不完全性 （2）室间隔膜部可见收缩）室间隔膜部可见收缩 期左室期左室右右 室红五彩分流束。室红五彩分流束。（3）仅一组共同瓣膜，各房室内血流）仅一组共同瓣膜，各房室内血流 互相混杂互相混杂（4）同时可见二、三尖瓣返流束）同时可见二、三尖瓣返流束（5）并肺高压时出现房、室水平的双向分）并肺高压时出现房、室水平的双向分流。流。1、Echoic discontinuity in inferior atrial spetum;shunt flow can be visualized in inferior atrial septu
28、m by Doppler ultrasound.2、With or without cleft of mitral/tricuspid anterior leaflet.3、Volume overloading of right ventricle may probably present。1、房间隔下部出现回声中断；多普勒超声、房间隔下部出现回声中断；多普勒超声 显示低位房间隔水平分流信号；显示低位房间隔水平分流信号；2、伴或不伴二、三尖瓣前叶裂隙。常伴右伴或不伴二、三尖瓣前叶裂隙。常伴右心容量负荷过重表现。心容量负荷过重表现。1、Echoic discontinuity in inferi
29、or atrial spetum and membranous ventricular septum,shunt flow can be visualized by Doppler ultrasound.2、Common undivided atrioventricular valve with cleft。3、Volume overloading of left and right ventricular may usually present。1、房间隔下部和室间隔膜部出现回声中断；、房间隔下部和室间隔膜部出现回声中断；多普勒超声显示上述部位有穿隔分流信号多普勒超声显示上述部位有穿隔分流信
30、号2、二、三尖瓣成共同房室瓣，并出现裂二、三尖瓣成共同房室瓣，并出现裂隙。隙。3、常伴左、右心容量负荷过重表现。、常伴左、右心容量负荷过重表现。Partial ECD Partial ECD OstiumOstium secundum atrial secundum atrial septal defect.septal defect.Complete ECD Complete ECD Single atrium,single Single atrium,single ventricle ventricle。部分型心内膜垫缺损：主要与部分型心内膜垫缺损：主要与继发孔房缺继发孔房缺鉴别。鉴别。完
31、全型心内膜垫缺损：主要与完全型心内膜垫缺损：主要与单心房、单单心房、单心室心室鉴别。鉴别。2D+CDFI ultrasound is the most valuable method2D technique searches for abnormalities in the atrioventricular valves,such as presence of cleft,searches for chordal attachment,overriding,or straddling of the valves.The crux of the heart is carefully analyz
32、ed by sweeping the transducer anterior to posterior to record the outlet and inlet portions of the membranous septum.CDFI techniques determine the direction and degree of regurgitation present in the atrioventricular valves and the direction of shunt flow.They help to determine operation strategy an
33、d evaluate the operation effect.二维超声结合彩色多普勒超声是目前确诊二维超声结合彩色多普勒超声是目前确诊本病的主要方法，能提供心内分流及瓣膜本病的主要方法，能提供心内分流及瓣膜返流的信息；并判别房、室间隔缺损的类返流的信息；并判别房、室间隔缺损的类型，为术式的选择提供重要依据，评价手型，为术式的选择提供重要依据，评价手术效果。术效果。1.By compare-expression teaching,We require students grip hemodynamics,ultracardiogram,diagnostic criterion,differen
34、tial diagnosis of PDA and ECD.2.We require students grip valuation of each ultracardiographic method,including two-dimensional-mode,motion-mode,color flow Doppler,spetral Dopple.and their findings of PDA and ECD.What is fetal circulation different from neonatal circulation?What transducer is the best to use for a fetal echocardiogram?What are the problems associated with a complete ECD?What is the difference between primum atrial septal defect and secondary atrial septum defect?
