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1、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,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Cardiac development during fetal period,The primi
2、tive heart tube forms by 3 W Cardiac looping by 2224 days Bend ventrally and toward the rightCardiac septation,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,Aortic arch development,Right and left truncoconal ridges The aortic and pulmonary outflow tracts are full
3、y separated The coronary sinusThe semilunar valves,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,正常血液循环途径,二 胎儿新生儿循环转换,The placenta:gas exchange,metabolic exchange The lung:constrictedThree cardiovascular structure:Ductus venosus Foram
4、en ovale Ductus arteriosus,The Fetal Circulation,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,The Fetal Circulation,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,Circulation Changes after birth,The umbilical cord clamped breathing commencesPulmo
5、nary circulation Providing gas exchange The foramen ovale and ductus arteriosus:functionally closed,胎儿与出生后血液循环比较,返回,Common symtoms,Congestive heart failureFeeding difficulties/Exercise intoleranceRespiratory distressPoor growthCracked,coughSquatting SyncopeCyanosis,三 儿童心血管疾病体格检查方法,Physical examinati
6、on,Commonly manifestationsEvaluation for BW and BH Respiratory distress Cyanosis Clubbing digits Hepatomegaly Distended neck veins Sign of liver-jugular vein Peripheral edema,Cardiovascular Examination,Inspection:Prominence of the precordium Cardiomegaly Palpation:heave,thrill Percussion:size and po
7、sition of the heartAuscultation:HR,rhythm(1)Normal heart sounds:S1,S2,S3,S4(2)Abnormal heart sounds:P2 fixed splitting(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)Location and radiation(2)Timing:Systolic,diastolic and continuous(3)Quality:Harsh,musical,or rough;high,medium or low in pitch(4)Int
8、ensity:grade,(5)Variation with position,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)Chest x-ray(2)Electrocardiogram(3)Echocardiogram(4)Cardiac catheterization(5)Angiocardiography(6)Radionuclide angiocardiography(7)Magnetic resonance imaging（8）Compute
9、d tomography,Chest Radiograph,character of the pulmonary vasculaturePosition of the cardiac apexcardiac size(cardiothoracic ratio)cardiac configuration,heart shapePosition of the abdominal viscera,Normal cardiothoracic imaging landmarks,PA view,Anterior radiographic view of cardiac anatomy,X-ray,The
10、 sequence of ECG:rate,rhythm,P wave,PR interval,QRS complex(axis,amplitude,and duration),QT interval,ST segment,and T wave.Assess atrioventricular hypertrophyDiagnosis of dysrhythmias,Electrocardiogram,ECG,Normal ECG,A powerful noninvasive method:M-mode,two and three dimensional,color and DopplerEva
11、luate cardiac structureEstimate intracardiac pressure and gradient across stenotic valves and vesselsQuantitate cardiac contractile functionDetermine the direction of flow across a defectExamine the integrity of the coronary arteriesEvaluate endocarditis,pericardiac fluid,cardiac tumors,or chamber t
12、hrombi.,Echocardiography,M-mode ECHO,Long-axis view for LV,2-D ECHO,Four-chamber view at apex,2-D ECHO,Short-axis view at the great artery,2-D color ECHO(four-chamber view),2-D color Doppler ECHO,3-D Reconstruction,SVC,IVC,RV,Cardiac Catheterization,Estimate Cardiac output according to Oxygen conten
13、t and saturation,pulmonary and systemic blood flow.Determine the pressures in all chambers and vesselsCalculate the pulmonary and systemic vascular resistanceInterventional catheterization(therapeutic procedures),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
14、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,心血管造影,心血管造影,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,Magnetic resonance imaging,Noninvasive toolFollow-upEvaluate the heart and the great arteriesProvide the imaging of vascular structures of the thoraxPaul C.Lauterbur,Uni
15、ted States,and Sir Peter Mansfield,Britain,wined the 2003 Nobel Prize for discoveries in magnetic resonance imaging,SE,AO,PA,AO,LV,LA,RV,Cine MRI,Cine-MRI,MRA,3-D MRI,MRI is in valuable in the long term follow-up of coarctation of the aorta after angioplasty.(surgical procedure),Computed tomography,
16、Follow-upEvaluate the heart and the great arteriesProvide the imaging of vascular structures of the thorax,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,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
17、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,Categories of congenital heart disease,The left-to-right shunt type(ASD VSD PDA),Acyanotic early Pulmonary blood increasedSystemic blood decreasedPulmonary hypertensionEisenmenger syndrome:cyanotic lately due to right-to-left shunts,The right-to-left shunt type,Tetr
18、alogy of Fallot(TOF),transposition of the great arteries(TGA)Cyanotic earlyCyanotic lesions with increased pulmonary blood flowCyanotic lesions with decreased pulmonary blood flowThe systemic circulation is hypoxemia,No-shunt lesions,Pulmonary stenosis(PS),Coarctation of the aorta(Coa)AcyanoticHyper
19、trophy resulting in increased pressure load,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”,Diagnostic process for CHD,Two groups based on PE acyanotic and cyanotic lesionsFurther be subdivided based on X-ray increased,normal or decreased pulmonary vascular markingsDetermin
20、e whether right,left,or biventricular hypertrophyConfirm the diagnosis by ECHO or Catheterization,or both,第三节 几种常见的先天性心脏病Common congenital heart diseases,Atrial Septal defect（ASD),ASD General considerations,Definition:an opening in the atrial septum permitting the shunting of blood between the atria
21、Incidence:10%in all CHD Three major types:(1)The ostium secundum type(2)The sinus venosus type(3)The ostium primum type,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 circu
22、lation,ASD Pathophysiology,In neonate and early infant limiting the L R shuntSmall ASDLarge ASD Qp:Qs 2 4:1PAH：R L shunt,ASD血液循环途径,Why the LA is not enlarged？,Small ASD：Most often asymptomatic discovered during PELarge ASD（large shunt）：(1)Pulmonry blood：repeated URI or pneumonia(2)Systemic blood：poo
23、r growth,pale,lack of power,hidrosis,tachypnea after exercise(3)Pulmonary hypertension,Eisenmengger syndrome（cyanosis）,ASD Clinical manifestation,ASD Cardiac sign,Inspection:left precordial bulge and increased cardiac activity Palpation:systolic lift,seldom with thrillPercussion:cardiomegalyAusculta
24、tion:loud S1 2,3 LSB systolic ejection murmur,grade fixed splitting of the 2nd heart sound Lower LSB diastolic murmur Early systolic click,P2 accentuated(PAH),P2亢进伴固定分裂，喀喇音,ASD-/SM,ASD ECG,Volume overload of the RV:QRS axis:normal or right axis deviation with a clockwise loop in the frontal planeRV
25、hypertrophy A minor right ventricular conduction delay P-R delay In the right precordial leads,a rsR pattern,ASD ECGI AVB,ASD X-ray,The pulmonary vascularity increasedEnlargement of the RV and RAThe pulmonary artery dilated,M mode:RV volume overload2-D：sizeColor：directionDoppler：pressure3-D：ASD 3-D,
26、ASD ECHO,ASD catheterization,Oxygen content in RA:higher The catheter:RA to LA through ASDThe PA pressure:usually normal pulmonary vascular resistance Qp:Qs:1.5:1 to 4:1.,ASD catheterization,ASD Prognosis and ComplicationsPneumoniaHeart failureInfective endocarditisPulmonary hypertensionAtrial arrhy
27、thmiaTricuspid regurgitation,ASD treatment,Surgical repair:26 yrsEarly surgery:Infants with CHF or significant pulmonary hypertension,“mini”sternotomy posterior thoracotomy approach Closure with device(Amplazer),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。,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,Closure procedure,
