齐鲁医学先天性心脏病英文.pptx

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1、1.Genetic factor(internalfactor):Genetic and chromosomal aberrations2.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 factorsEtiology12021/7/27 星期二The

2、health protection of pregnant woman should be enhanced.High risk factors,such as drugs,radiation,viral infection,et.should be avoided.Suit dosage Folic Acid should be filled up in early pregnancy stage.Prevention22021/7/27 星期二Does the child have heart disease?Evaluating a child with a heart murmurIs

3、 it congenital heart disease?If it is congenital heart disease,what is the lesion?What is the severity of the lesion?32021/7/27 星期二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 fa

4、ilureMinor1.Systolic murmur less than grade III in intensity 2.Abnormal S23.Abnormal ECG4.Abnormal X-ray5.Abnormal BP42021/7/27 星期二CHDCHDAcyanotic Acyanotic CHDCHDCyanoticCyanoticCHDCHDL to R shuntsL to R shuntsASD ASD VSDVSDPDA PDA Obstructive lesions Pulmonic stenosisAortic stenosisCoarctation of

5、aortaMitral regurgitationR to L shuntsR to L shuntsTOFTOFComplete TGAComplete TGA52021/7/27 星期二TOFPDAVSDASD Four Congenital Cardiac Anomalies in Children 返回返回62021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial Septal Defect(ASD)ASD is the most frequent congenital lesion of major importance

6、 in adults.It is often not diagnosed until adult life,even in the present era,because it rarely produces symptoms in childhood and the associated physical signs are easily confused with the cardiac findings in normal children.72021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial Septal Defec

7、t Three types of atrial septal defect are classified on an anatomic basis:ostium 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.82021/7/27 星期二92021/7/27 星期二房间隔缺损房间隔缺损102021/7/27 星

8、期二房间隔缺损房间隔缺损原发孔型原发孔型位于房间隔位于房间隔的下部，紧的下部，紧邻房室瓣邻房室瓣。112021/7/27 星期二房间隔缺损房间隔缺损继发孔型继发孔型亦称中央型亦称中央型或卵圆孔型或卵圆孔型122021/7/27 星期二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132021/7/27 星期二房间隔缺损房间隔缺损142021/7/27 星期二152021/7/27 星期二162021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial Septal Defect Blood is chronica

9、lly overcirculated through the lungs at normal intracardiac pressure levels.Increased flow through 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 split

10、ting of the second heart sound,the other classic finding in ASD.172021/7/27 星期二Congenital Cardiac 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 usua

11、lly reveals enlargement of the heart and signs of pulmonary overcirculation,such as a large pulmonary trunk and increased pulmonary vascular markings.The relative severity of these conditions reflects the size of the left-to-right shunt.182021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial

12、Septal Defect Two major complications of ASD are pulmonary arterial hypertension and right ventricular 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 plexi

13、form arteriopathy丛状的动脉病,similar to that seen in Eisenmenger syndrome or primary pulmonary hypertension,is present.192021/7/27 星期二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

14、finally reverses;a right ventricular pressure overload develops,pulmonary blood flow is reduced,and the patient becomes cyanotic.202021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial Septal Defect Right ventricular failure develops as a result of long-standing volume overload;it usually aff

15、ects patients older than 40 years.Right ventricular failure is usually associated with atrial flutter 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

16、clinically between ASD and such conditions as cardiomyopathy and mitral valve disease.212021/7/27 星期二Congenital 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

17、the ratio of pulmonary blood flow to systemic blood flow is 2:1 or greater.Nearly all patients in whom ASD can be clinically diagnosed exhibit at least this degree of left-to-right shunt.222021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial Septal Defect Surgery is contraindicated 治疗或处置不当 w

18、hen pulmonary hypertension approaches the pressure level of the systemic circulation because in such patients the operative mortality is high and the elevated pulmonary vascular resistance does not fall after surgery.232021/7/27 星期二Congenital Cardiac Anomalies in Children Atrial Septal Defect Nonsur

19、gical closure using cardiac catheterization with an umbrellalike device has been accomplished in patients with defects less than 2 cm in diameter,most of whom have been infants or small children.242021/7/27 星期二房间隔缺损封堵伞房间隔缺损封堵伞252021/7/27 星期二房间隔缺损封堵过程房间隔缺损封堵过程262021/7/27 星期二Congenital Cardiac Anomali

20、es in Children Ventricular Septal Defect(VSD)VSD is the most common congenital cardiac anomaly in infants.It 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 h

21、igh;closure occurs particularly often in infancy but also in later years.272021/7/27 星期二282021/7/27 星期二VSD292021/7/27 星期二VSD302021/7/27 星期二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.

22、Because the opening is quite small,normal systolic pressure can be maintained in the right ventricle and in the pulmonary artery.312021/7/27 星期二Congenital Cardiac Anomalies in Children Ventricular Septal Defect In infants with a large VSD,medical management has two aims:to control heart failure and

23、to prevent the development of pulmonary vascular disease.Therapeutic measures are aimed at the control of heart failure symptoms and the maintenance of normal growth.322021/7/27 星期二Congenital Cardiac Anomalies in Children Ventricular Septal Defect Indications for surgical closure of VSD include pati

24、ents at any age with large defects in whom clinical symptoms and failure to thrive cannot be controlled medically.Infants between 6 and 12 mo of age with large defects associated with pulmonary hypertension,even if symptoms are controlled by medication.332021/7/27 星期二Congenital Cardiac Anomalies in

25、Children Ventricular Septal Defect Surgical closure is usually undertaken to prevent infective endocarditis.The incidence of this complication is not well established,but surgery appears to be highly effective as a prophylactic measure.342021/7/27 星期二室间隔缺损封堵伞室间隔缺损封堵伞352021/7/27 星期二室间隔缺损封堵室间隔缺损封堵3620

26、21/7/27 星期二Patent Ductus ArteriosusPDA Persistence of the normal fetal vessel that joins the PA to the Aorta.Normally closes 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 as

27、sociated w/coarctation&VSD.372021/7/27 星期二动脉导管未闭动脉导管未闭PDA382021/7/27 星期二Patent Ductus ArteriosusQuestion:What TORCH infection is PDA associated with?Answer:Rubella392021/7/27 星期二Patent Ductus ArteriosusHemodynamicsAs a result of higher aortic pressure,blood shunts L to R through the ductus from Aort

28、a to PA.Extent of the shunt depends on size of the ductus&PVR:SVR.Small PDA,pressures in PA,RV,RA are normal.402021/7/27 星期二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.Lead

29、s to increased pulmonary vascular disease.412021/7/27 星期二Patent Ductus ArteriosusClinical Signs&SymptomsSmall PDAs are usually asymptomaticLarge PDAs can result in symptoms of CHF,growth restriction,FTT.Bounding arterial pulsesWidened pulse pressure Enlarged heart,prominent apical impulseClassic con

30、tinuous machinary systolic murmurMid-diastolic murmur at the apex422021/7/27 星期二Patent Ductus ArteriosusTreatmentIndomethacin,inhibitor of prostaglandin synthesis can be used in premature infants.PDA requires surgical or catheter closure.Closure is required treatment heart failure&to prevent pulmona

31、ry vascular disease.Usually done by ligation&division or intra vascular coil.Mortality is 1%432021/7/27 星期二动脉导管未闭封堵伞动脉导管未闭封堵伞442021/7/27 星期二法洛四联症法洛四联症紫绀型紫绀型452021/7/27 星期二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 space462021/7/27 星期二