1、CongenitalHeartDiseasewan.EnglishisalonCongenital 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.Overview1.Genetic factor(internalfactor):Genetic and chromosomal a
2、berrations2.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 factorsEtiologyThe health protection of pregnant woman should be enhanced.High risk factors
3、,such as drugs,radiation,viral infection,et.should be avoided.Suit dosage Folic Acid should be filled up in early pregnancy stage.PreventionDoes the child have heart disease?Evaluating a child with a heart murmurIs it congenital heart disease?If it is congenital heart disease,what is the lesion?What
4、 is the severity of the lesion?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 S23.Abnormal ECG4.Abnormal X
5、-ray5.Abnormal BPCHDCHDAcyanotic 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 TGAComplete TGATOFPDAVSDASD Four Congenital Card
6、iac Anomalies in Children 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,because it rarely produces symptoms in childhood and the associated
7、physical signs are easily confused with the cardiac findings in normal children.Congenital Cardiac Anomalies in Children Atrial Septal Defect 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 w
8、ith a left-to-right shunt at the atrial level and volume overwork of the right ventricle.房间隔缺损房间隔缺损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。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房间隔缺损房间隔缺损Congenital Cardiac Anomalies in Children Atrial Septal Defect Blood is c
9、hronically 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 wid
10、e splitting of the second heart sound,the other classic finding in ASD.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 usually reve
11、als 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.Congenital Cardiac Anomalies in Children Atrial Septal Defect Two major
12、 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 plexiform arteriopathy丛状的动脉病
13、,similar to that seen in Eisenmenger syndrome or primary pulmonary hypertension,is present.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 p
14、ressure overload develops,pulmonary blood flow is reduced,and the patient becomes cyanotic.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 f
15、ailure 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 clinically between ASD and such conditions as cardiom
16、yopathy and mitral valve disease.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 the ratio of pulmonary blood flow to systemic blood flow is 2:1 or g
17、reater.Nearly all patients in whom ASD can be clinically diagnosed exhibit at least this degree of left-to-right shunt.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 circulatio
18、n because in such patients the operative mortality is high and the elevated pulmonary vascular resistance does not fall after surgery.Congenital Cardiac Anomalies in Children Atrial Septal Defect N o n s u r g i c a l c l o s u r e u s i n g c a r d i a c catheterization with an umbrellalike device
19、has been accomplished in patients with defects less than 2 cm in diameter,most of whom have been infants or small children.房间隔缺损封堵伞房间隔缺损封堵伞房间隔缺损封堵过程房间隔缺损封堵过程Congenital Cardiac Anomalies in Children Ventricular Septal Defect(VSD)VSD is the most common congenital cardiac anomaly in infants.It is rarel
20、y 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.VSDVSDCongenital Cardiac Anomalies in Childr
21、en 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 pulmonary artery.Congenital Cardiac Anomalies in Children Ventric
22、ular 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 failure symptoms and the maintenance of normal growth.Congenital Cardiac Anomali
23、es 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.Infants between 6 and 12 mo of age with large defects associated with pulmonary hypertension,
24、even if symptoms are controlled by medication.Congenital Cardiac Anomalies in 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 prophyl
25、actic measure.室间隔缺损封堵伞室间隔缺损封堵伞室间隔缺损封堵室间隔缺损封堵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
26、.Female:Male ratio of 2:1Often associated w/coarctation&VSD.动脉导管未闭动脉导管未闭PDAPatent Ductus ArteriosusQuestion:What TORCH infection is PDA associated with?Answer:RubellaPatent Ductus ArteriosusHemodynamicsAs a result of higher aortic pressure,blood shunts L to R through the ductus from Aorta to PA.Exte
27、nt of the shunt depends on size of the ductus&PVR:SVR.Small PDA,pressures in PA,RV,RA are normal.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 va
28、scular disease.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 continuous machinary systolic murmurMid-diast
29、olic murmur at the apexPatent 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 pulmonary vascular disease.Usually done by ligation&division or
30、intra vascular coil.Mortality is 1%动脉导管未闭封堵伞动脉导管未闭封堵伞法洛四联症法洛四联症紫绀型紫绀型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 spaceWhat is this disease?Atrial Septal Defect 谢谢！
