,*,庞玉生,授课对象：本科（七年制）,广西医科大学儿科学教研室,Chapter 13 循环系统疾病,Circulation system disease,庞玉生授课对象：本科（七年制）Chapter 13 循环系统,1,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,教学目的,2,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,Contents小儿心血管系统解剖生理特点及检查方法,3,第一节 小儿心血管系统解剖生理 特点及检查方法,第一节 小儿心血管系统解剖生理 特点及检查方法,4,一 心脏的胚胎发育,Cardiac development during fetal period,The primitive heart tube forms by,3 W,Cardiac looping by,2224 days,Bend ventrally and toward the right,Cardiac septation,一 心脏的胚胎发育Cardiac development,5,第一隔,原发孔,心内膜垫,静脉窦瓣膜,第二隔,继发孔,第一隔,室间孔,第二隔,继发孔,第一隔,卵园孔,第一隔,室间孔,第二隔,静脉窦瓣膜,胚胎房室分隔及房室间隔发育,第一隔原发孔心内膜垫静脉窦瓣膜第二隔继发孔第一隔室间孔第二隔,6,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,室间隔形成 心室间隔组成三个来源 肌隔,7,Aortic arch development,Right and left truncoconal ridges,The aortic and pulmonary outflow tracts are fully separated,The coronary sinus,The semilunar valves,Aortic arch developmentRight a,8,主肺动脉由总干呈螺旋形分隔,AO,PA,PA,AO,PA,PA,AO,AO,主肺动脉由总干呈螺旋形分隔AOPAPAAOPAPAAOAO,9,P,A,P,A,P,A,P,A,大动脉位置变移系列示意图,TOF,TGA,DORV,Taussig-Bing,PAPAPAPA大动脉位置变移系列示意图TOFTGADORV,10,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,房室管、心房、室间隔膜部的形成及室间隔的封闭第2周原始心脏,11,正常血液循环途径,正常血液循环途径,12,二 胎儿新生儿循环转换,二 胎儿新生儿循环转换,13,The placenta:,gas exchange, metabolic exchange,The lung: constricted,Three cardiovascular structure:,D,uctus venosus,Foramen ovale,Ductus arteriosus,The Fetal Circulation,The placenta:The Fetal Circula,14,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,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,15,The Fetal Circulation,The Fetal Circulation,16,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,下腔静脉,右心房,右心室,左心房,左心室,升主动脉,心脑及上肢,肺动脉,降主动脉,下半身,上半身静脉血,上腔静脉,脐静脉动脉血,门静脉静脉血,母体,下半身静脉血,肺循环,50%,1/3,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,17,Circulation Changes after birth,The umbilical cord clamped,breathing commences,Pulmonary circulation,Providing gas exchange,The foramen ovale and ductus arteriosus: functionally closed,Circulation Changes after birt,18,胎儿与出生后血液循环比较,A胎儿期,B出生后,由母体循环完成气体交换,由肺循环完成气体交换,多为混合血，心、脑、上半身血氧含量高于下半身,静脉血和动脉血分开,卵圆孔、动脉导管、静脉导管开放,卵圆孔、动脉导管、静脉导管闭合,肺动脉压与主动脉相似，肺循环阻力高,肺动脉压下降，肺循环阻力低,右心室高负荷,左心室高负荷,A,B,返回,胎儿与出生后血液循环比较A胎儿期B出生后由母体循环完成气体交,19,Common symtoms,Congestive heart failure,Feeding difficulties / Exercise intolerance,Respiratory distress,Poor growth,Cracked, cough,Squatting,Syncope,Cyanosis,Common symtoms Congestive hear,20,三 儿童心血管疾病体格检查方法,三 儿童心血管疾病体格检查方法,21,Physical examination,Commonly manifestations,Evaluation for BW and BH,Respiratory distress,Cyanosis,Clubbing digits,Hepatomegaly,Distended neck veins,Sign of liver -jugular vein,Peripheral edema,Physical examinationCommonly m,22,Cardiovascular Examination,Inspection:,Prominence of the precordium,Cardiomegaly,Palpation:,heave, thrill,Percussion:,size and position of the heart,Auscultation:,HR, rhythm,(1) Normal heart sounds: S1, S2, S3, S4,(2) Abnormal heart sounds: P2 fixed splitting,(3) Murmurs,Cardiovascular ExaminationInsp,23,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,小儿听诊常用听诊区,24,(1) Location and radiation,(2) Timing: Systolic, diastolic and continuous,(3) Quality: Harsh, musical, or rough; high, medium or low in pitch,(4) Intensity: grade , , , , , ,(5) Variation with position,Murmur,(1) Location and radiationMurm,25,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,周围血管征四肢脉搏及血压,26,四 心脏的特殊检查,(1) Chest x-ray,(2) Electrocardiogram,(3) Echocardiogram,(4) Cardiac catheterization,(5) Angiocardiography,(6) Radionuclide angiocardiography,(7) Magnetic resonance imaging,（8）Computed tomography,四 心脏的特殊检查,27,Chest Radiograph,character of the pulmonary vasculature,Position of the cardiac apex,cardiac size (cardiothoracic ratio),cardiac configuration, heart shape,Position of the abdominal viscera,Chest Radiographcharacter of t,28,先心双语(七年制)_医学医药ppt课件,29,Normal cardiothoracic imaging landmarks, PA view,Normal cardiothoracic imaging,30,Anterior radiographic view of cardiac anatomy,Anterior radiographic view of,31,X-ray,X-ray,32,你的X线诊断分别是什么？,你的X线诊断分别是什么？,33,The sequence of ECG:,rate, rhythm, P wave, PR interval, QRS complex ( axis, amplitude, and duration), QT interval, ST segment, and T wave.,Assess atrioventricular hypertrophy,Diagnosis of dysrhythmias,Electrocardiogram, ECG,The sequence of ECG: Electroca,34,先心双语(七年制)_医学医药ppt课件,35,Normal ECG,Normal ECG,36,A powerful noninvasive method: M-mode, two and three dimensional, color and Doppler,Evaluate cardiac structure,Estimate intracardiac pressure and gradient across stenotic valves and vessels,Quantitate cardiac contractile function,Determine the direction of flow across a defect,Examine the integrity of the coronary arteries,Evaluate endocarditis, pericardiac fluid, cardiac tumors, or chamber thrombi.,Echocardiography,A powerful noninvasive method:,37,先心双语(七年制)_医学医药ppt课件,38,M-mode ECHO,M-mode ECHO,39,Long-axis view for LV,2-D ECHO,Long-axis view for LV2-D ECHO,40,Four-chamber view at apex,2-D ECHO,Four-chamber view at apex2-D E,41,Short-axis view at the great artery,Short-axis view at the great a,42,2-D color ECHO (four-chamber view),2-D color ECHO (four-chamber v,43,2-D color Doppler ECHO,2-D color Doppler ECHO,44,3-D Reconstruction,3-D Reconstruction,45,SVC,IVC,RV,SVCIVCRV,46,Cardiac Catheterization,Estimate Cardiac output according to Oxygen content and saturation, pulmonary and systemic blood flow.,Determine the pressures in all chambers and vessels,Calculate the pulmonary and systemic vascular resistance,Interventional catheterization (therapeutic procedures),20%,1214%,30/12,1214%,1214%,4 8,0 5,100/10,30/5,100/60,Cardiac CatheterizationEstimat,47,先心双语(七年制)_医学医药ppt课件,48,Angiocardiography,选择性造影,Selective angiocardiography,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6075向头成角2030 ,肝锁位：左前斜4045向头成角40 ,坐观位：正位向头成角40 ,Angiocardiography选择性造影 Selecti,49,心血管造影,心血管造影,50,心血管造影,心血管造影,51,Radionuclide angiocardiography,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,Radionuclide angiocardiography,52,Magnetic resonance imaging,Noninvasive tool,Follow-up,Evaluate the heart and the great arteries,Provide the imaging of vascular structures of the thorax,Paul C. Lauterbur, United States, and Sir Peter Mansfield, Britain, wined the 2003 Nobel Prize for discoveries in magnetic resonance imaging,Magnetic resonance imagingNoni,53,SE,SE,54,AO,PA,AOPA,55,AO,LV,LA,RV,Cine MRI,AOLVLARVCine MRI,56,Cine-MRI,Cine-MRI,57,MRA,MRA,58,3-D MRI,MRI is in valuable in the long term follow-up of coarctation of the aorta after angioplasty. (surgical procedure),3-D MRIMRI is in valuable in t,59,Computed tomography,Follow-up,Evaluate the heart and the great arteries,Provide the imaging of vascular structures of the thorax,Computed tomographyFollow-up,60,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:68，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,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,61,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,病 因 内因：与遗传有关，染色体异常或基因突变。,62,Categories of congenital heart disease,Categories of congenital heart,63,The left-to-right shunt type,(ASD VSD PDA),Acyanotic early,Pulmonary blood increased,Systemic blood decreased,Pulmonary hypertension,Eisenmenger syndrome: cyanotic lately due to right-to-left shunts,The left-to-right shunt typeAc,64,The right-to-left shunt type,Tetralogy of Fallot ( TOF), transposition of the great arteries (TGA),Cyanotic early,Cyanotic lesions with increased pulmonary blood flow,Cyanotic lesions with decreased pulmonary blood flow,The systemic circulation is hypoxemia,The right-to-left shunt typeTe,65,No-shunt lesions,Pulmonary stenosis (PS),Coarctation of the aorta (Coa),Acyanotic,Hypertrophy resulting in increased pressure load,No-shunt lesionsPulmonary sten,66,Van Praagh 顺序分段诊断方法,心房位置判断,心室位置判断,大血管位置判断,房室连接诊断,心室大动脉连接诊断,心脏位置,“三层楼结构”,“四层楼结构”,Van Praagh 顺序分段诊断方法心房位置判断“三层楼结,67,Diagnostic process for CHD,Two groups based on PE,acyanotic and cyanotic lesions,Further be subdivided based on X-ray increased, normal or decreased pulmonary vascular markings,Determine whether right, left, or biventricular hypertrophy,Confirm the diagnosis by ECHO or Catheterization, or both,Diagnostic process for CHDTwo,68,第三节 几种常见的先天性心脏病,Common congenital heart diseases,第三节 几种常见的先天性心脏病,69,先心双语(七年制)_医学医药ppt课件,70,Atrial Septal defect,（ASD),Atrial Septal defect （ASD),71,ASD General considerations,Definition: an opening in the atrial septum permitting the shunting of blood between the atria,Incidence: 10% in all CHD,Three major types:,(1) The ostium secundum type,(2) The sinus venosus type,(3) The ostium primum type,ASD General considerationsDefi,72,ASD,Pathophysiology,The degree of L-R shunt,(1) The size of the defect,(2) The relative compliances of RV and LV,(3) the relative vascular resistances in the pulmonary and systemic circulation,ASD PathophysiologyThe degree,73,ASD Pathophysiology,In neonate and early infant,limiting the L R shunt,Small ASD,Large ASD,Qp:Qs 2 4:1,PAH：R L shunt,ASD PathophysiologyIn neonate,74,可在临床上表现出来,ASD分流,右心室扩大,肺动脉充血,肺循环充血,肺动脉高压,肺静脉动脉血,左心房,左心室,主动脉搏血减少,体循环缺血,上下腔静脉,右心房扩大,ASD,血液循环途径,可在临床上表现出来ASD分流右心室扩大肺动脉充血肺循环充血肺,75,LV,RV,LA,RA,Why the LA is not enlarged ？,Why the LA is not enlarged ？,76,Small ASD：,Most,often asymptomatic discovered during PE,Large ASD（large shunt）：,(1)Pulmonry blood ：repeated URI or pneumonia,(2)Systemic blood ：poor growth, pale, lack of power, hidrosis, tachypnea after exercise,(3)Pulmonary hypertension, Eisenmengger syndrome,（cyanosis）,ASD Clinical manifestation,Small ASD： Most often asymptom,77,ASD Cardiac sign,Inspection:,left precordial bulge,and increased cardiac activity,Palpation:,systolic lift,seldom with thrill,Percussion:,cardiomegaly,Auscultation:,loud S1,2,3 LSB,systolic ejection murmur,grade ,fixed splitting of the 2,nd,heart sound,Lower LSB diastolic murmur,Early systolic click , P2 accentuated (PAH),ASD Cardiac signInspection: le,78,P2亢进伴固定分裂，喀喇音,ASD -,/ SM,P2亢进伴固定分裂，喀喇音ASD -/ SM,79,ASD ECG,Volume overload of the RV:,QRS axis: normal or right axis deviation,with a clockwise loop in the frontal plane,RV hypertrophy,A minor right ventricular conduction delay,P-R delay,In the right precordial leads, a rsR pattern,ASD ECGVolume overload of the,80,ASD ECG,I AVB,ASD ECGI AVB,81,ASD X-ray,The pulmonary vascularity increased,Enlargement of the RV and RA,The pulmonary artery dilated,ASD X-rayThe pulmonary vascu,82,M mode:,RV volume overload,2-D：,size,Color：,direction,Doppler：,pressure,3-D：,ASD 3-D,ASD ECHO,M mode:ASD ECHO,83,ASD catheterization,Oxygen content in RA: higher,The catheter : RA to LA through ASD,The PA pressure: usually normal,pulmonary vascular resistance,Qp:Qs:,1.5:1 to 4:1.,ASD catheterization Oxy,84,ASD catheterization,ASD catheterization,85,ASD Prognosis and Complications,Pneumonia,Heart failure,Infective endocarditis,Pulmonary hypertension,Atrial arrhythmia,Tricuspid regurgitation,ASD Prognosis and Complication,86,ASD treatment,Surgical repair: 26 yrs,Early surgery: Infants with CHF or significant pulmonary hypertension,“mini” sternotomy,posterior thoracotomy approach,Closure with device (Amplazer),ASD treatmentSurgical repair:,87,ASD,封堵适应证(,Amplatzer),年龄：通常3岁，8kg,继发孔型左向右分流ASD,直径5mm,房间隔的伸展径大于房缺直径14mm以上,不合并必须外科手术的其他心脏畸形,外科术后残余分流,ASD封堵适应证(Amplatzer)年龄：通常3岁，6,88,ASD封堵禁忌症,明显紫绀，肺动脉高压，右向左分流；,部分或完全肺静脉畸形引流；,筛网状、冠状窦型及多发ASD；,左心房发育不良；,复杂型先天性心脏病；,全身感染，出凝血功能障碍，阿司匹林禁忌。,ASD封堵禁忌症明显紫绀，肺动脉高压，右向左分流；,89,自膨胀双伞镍钛合金网结构；,左房侧比右房侧大4mm；,腰部为3层膜结构，直径即封堵器大小，腰长34mm。,ASD封堵器,自膨胀双伞镍钛合金网结构；ASD封堵器,90,Closure procedure,Closure procedure,91,