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1、先天性心脏病英文第一页，讲稿共五十一页哦Congenital heart disease are abnormalities in the hearts structure that are present at birth.Approximately 8 out of every 1,000 newborns have congenital heart defects,ranging from mild to severe.Overview第二页，讲稿共五十一页哦1.Genetic factor(internalfactor):Genetic and chromosomal aberrati
2、ons2.Environmental factor(external factor):High altitude4.Inherited factor3.Other related factors:Viral infections of pregnancy,Mothers who are diabetic,alcoholics or drug addictive Drugs and metabolic factorsEtiology第三页，讲稿共五十一页哦The health protection of pregnant woman should be enhanced.High risk fa
3、ctors,such as drugs,radiation,viral infection,et.should be avoided.Suit dosage Folic Acid should be filled up in early pregnancy stage.Prevention第四页，讲稿共五十一页哦Does the child have heart disease?Evaluating a child with a heart murmurIs it congenital heart disease?If it is congenital heart disease,what i
4、s the lesion?What is the severity of the lesion?第五页，讲稿共五十一页哦Assessment of a child for the presence of heart diseaseMajor 1.Systolic murmur garde III or more specially with a thrill2.Diastolic murmur3.Cyanosis4.Congestive heart failureMinor1.Systolic murmur less than grade III in intensity 2.Abnormal
5、 S23.Abnormal ECG4.Abnormal X-ray5.Abnormal BP第六页，讲稿共五十一页哦CHDCHDAcyanotic Acyanotic CHDCHDCyanoticCyanoticCHDCHDL to R shuntsL to R shuntsASD ASD VSDVSDPDA PDA Obstructive lesions Pulmonic stenosisAortic stenosisCoarctation of aortaMitral regurgitationR to L shuntsR to L shuntsTOFTOFComplete TGAComp
6、lete TGA第七页，讲稿共五十一页哦TOFPDAVSDASD Four Congenital Cardiac Anomalies in Children 返回返回第八页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect(ASD)ASD is the most frequent congenital lesion of major importance in adults.It is often not diagnosed until adult life,even in the present era
7、,because it rarely produces symptoms in childhood and the associated physical signs are easily confused with the cardiac findings in normal children.第九页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect Three types of atrial septal defect are classified on an anatomic basis:ostiu
8、m secundum第二孔,sinus venosus,and ostium primum第一孔.All three types are associated with a left-to-right shunt at the atrial level and volume overwork of the right ventricle.第十页，讲稿共五十一页哦第十一页，讲稿共五十一页哦房间隔缺损房间隔缺损第十二页，讲稿共五十一页哦房间隔缺损房间隔缺损原发孔型原发孔型位于房间隔的位于房间隔的下部，紧邻房下部，紧邻房室瓣室瓣。第十三页，讲稿共五十一页哦房间隔缺损房间隔缺损继发孔型继发孔型亦称中央
9、型或亦称中央型或卵圆孔型卵圆孔型第十四页，讲稿共五十一页哦房间隔缺损房间隔缺损静脉窦型静脉窦型位置接近上位置接近上腔静脉腔静脉第十五页，讲稿共五十一页哦房间隔缺损房间隔缺损第十六页，讲稿共五十一页哦第十七页，讲稿共五十一页哦第十八页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect Blood is chronically overcirculated through the lungs at normal intracardiac pressure levels.Increased flow throu
10、gh the pulmonary valve produces a characteristic pulmonary systolic ejection murmur.The pulmonary valve closes late because of the reduced impedance阻抗 in the pulmonary arterial system,causing a wide splitting of the second heart sound,the other classic finding in ASD.第十九页，讲稿共五十一页哦Congenital Cardiac
11、Anomalies in Children Atrial Septal Defect The splitting remains relatively fixed in relation to respiration;the aortic and pulmonary components remain audibly split during expiration.A chest x-ray usually reveals enlargement of the heart and signs of pulmonary overcirculation,such as a large pulmon
12、ary trunk and increased pulmonary vascular markings.The relative severity of these conditions reflects the size of the left-to-right shunt.第二十页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect Two major complications of ASD are pulmonary arterial hypertension and right ventricul
13、ar failure.Pulmonary arterial hypertension is caused by elevated pulmonary vascular resistance;it develops after adolescence in about 15 percent of cases.In the most severe cases,an irreversible plexiform arteriopathy丛状的动脉病,similar to that seen in Eisenmenger syndrome or primary pulmonary hypertensi
14、on,is present.第二十一页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect As a result of pulmonary hypertension,the left-to-right shunt first decreases,then becomes bidirectional,and finally reverses;a right ventricular pressure overload develops,pulmonary blood flow is reduced,and t
15、he patient becomes cyanotic.第二十二页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect Right ventricular failure develops as a result of long-standing volume overload;it usually affects patients older than 40 years.Right ventricular failure is usually associated with atrial flutter
16、or fibrillation and is often linked to tricuspid regurgitation.Eventually,a syndrome of right-and left-sided congestive heart failure develops,and at this stage,it may be difficult to differentiate clinically between ASD and such conditions as cardiomyopathy and mitral valve disease.第二十三页，讲稿共五十一页哦Co
17、ngenital Cardiac Anomalies in Children Atrial Septal Defect Surgical closure of ASD is a very safe and highly effective procedure.Prophylactic surgery is therefore indicated in any patient in whom the ratio of pulmonary blood flow to systemic blood flow is 2:1 or greater.Nearly all patients in whom
18、ASD can be clinically diagnosed exhibit at least this degree of left-to-right shunt.第二十四页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect Surgery is contraindicated 治疗或处置不当 when pulmonary hypertension approaches the pressure level of the systemic circulation because in such pat
19、ients the operative mortality is high and the elevated pulmonary vascular resistance does not fall after surgery.第二十五页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Atrial Septal Defect Nonsurgical closure using cardiac catheterization with an umbrellalike device has been accomplished in patients
20、 with defects less than 2 cm in diameter,most of whom have been infants or small children.第二十六页，讲稿共五十一页哦房间隔缺损封堵伞房间隔缺损封堵伞第二十七页，讲稿共五十一页哦房间隔缺损封堵过程房间隔缺损封堵过程第二十八页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Ventricular Septal Defect(VSD)VSD is the most common congenital cardiac anomaly in infants.It
21、 is rarely seen in adults because substantial VSD that are not corrected surgically are associated with a high mortality.In addition,the incidence of spontaneous closure of VSD is relatively high;closure occurs particularly often in infancy but also in later years.第二十九页，讲稿共五十一页哦第三十页，讲稿共五十一页哦VSD第三十一页
22、，讲稿共五十一页哦VSD第三十二页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Ventricular Septal Defect The VSD that do appear in adults as isolated anomalies are usually less than 1 cm in diameter.Because the opening is quite small,normal systolic pressure can be maintained in the right ventricle and in the p
23、ulmonary artery.第三十三页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Ventricular Septal Defect In infants with a large VSD,medical management has two aims:to control heart failure and to prevent the development of pulmonary vascular disease.Therapeutic measures are aimed at the control of heart fa
24、ilure symptoms and the maintenance of normal growth.第三十四页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Ventricular Septal Defect Indications for surgical closure of VSD include patients at any age with large defects in whom clinical symptoms and failure to thrive cannot be controlled medically.I
25、nfants between 6 and 12 mo of age with large defects associated with pulmonary hypertension,even if symptoms are controlled by medication.第三十五页，讲稿共五十一页哦Congenital Cardiac Anomalies in Children Ventricular Septal Defect Surgical closure is usually undertaken to prevent infective endocarditis.The inci
26、dence of this complication is not well established,but surgery appears to be highly effective as a prophylactic measure.第三十六页，讲稿共五十一页哦室间隔缺损封堵伞室间隔缺损封堵伞第三十七页，讲稿共五十一页哦室间隔缺损封堵室间隔缺损封堵第三十八页，讲稿共五十一页哦Patent Ductus ArteriosusPDA Persistence of the normal fetal vessel that joins the PA to the Aorta.Normally c
27、loses in the 1st wk of life.Accounts for 10%of all CHD,seen in 10%of other congenital hrt lesions and can often play a critical role in some lesions.Female:Male ratio of 2:1Often associated w/coarctation&VSD.第三十九页，讲稿共五十一页哦动脉导管未闭动脉导管未闭PDA第四十页，讲稿共五十一页哦Patent Ductus ArteriosusQuestion:What TORCH infect
28、ion is PDA associated with?Answer:Rubella第四十一页，讲稿共五十一页哦Patent Ductus ArteriosusHemodynamicsAs a result of higher aortic pressure,blood shunts L to R through the ductus from Aorta to PA.Extent of the shunt depends on size of the ductus&PVR:SVR.Small PDA,pressures in PA,RV,RA are normal.第四十二页，讲稿共五十一页哦
29、Patent Ductus ArteriosusHemodynamicsLarge PDA,PA pressures are equal to systemic pressures.In extreme cases 70%of CO is shunted through the ductus to pulmonary circulation.Leads to increased pulmonary vascular disease.第四十三页，讲稿共五十一页哦Patent Ductus ArteriosusClinical Signs&SymptomsSmall PDAs are usuall
30、y asymptomaticLarge PDAs can result in symptoms of CHF,growth restriction,FTT.Bounding arterial pulsesWidened pulse pressure Enlarged heart,prominent apical impulseClassic continuous machinary systolic murmurMid-diastolic murmur at the apex第四十四页，讲稿共五十一页哦Patent Ductus ArteriosusTreatmentIndomethacin,
31、inhibitor of prostaglandin synthesis can be used in premature infants.PDA requires surgical or catheter closure.Closure is required treatment heart failure&to prevent pulmonary vascular disease.Usually done by ligation&division or intra vascular coil.Mortality is 1%第四十五页，讲稿共五十一页哦动脉导管未闭封堵伞动脉导管未闭封堵伞第四
32、十六页，讲稿共五十一页哦法洛四联症法洛四联症紫绀型紫绀型第四十七页，讲稿共五十一页哦Case reportOne girl with 6 year oldSymptoms:recurrent chest infections/wheezePhysical signsA fixed and widely split second heart soundAn ejection systolic murmur best heard in the third left intercostal space第四十八页，讲稿共五十一页哦What is this disease?Atrial Septal Defect第四十九页，讲稿共五十一页哦第五十页，讲稿共五十一页哦 谢谢！第五十一页，讲稿共五十一页哦