先天性心脏病(英文).ppt

1、Congenital Heart Disease,wan.Englishi salon,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,1.Genetic factor (internalfactor) :Genetic and ch

2、romosomal aberrations,2. Environmental factor (external factor): High altitude,4. Inherited factor,3. Other related factors: Viral infections of pregnancy , Mothers who are diabetic, alcoholics or drug addictive Drugs and metabolic factors,Etiology,The health protection of pregnant woman should be e

3、nhanced. 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.,Prevention,Does the child have heart disease?,Evaluating a child with a heart murmur,Is it congenital heart disease?,If it is congenital h

4、eart disease, what is the lesion?,What is the severity of the lesion?,Assessment of a child for the presence of heart disease,Major Systolic murmur garde III or more specially with a thrill Diastolic murmur Cyanosis Congestive heart failure,Minor,Systolic murmur less than grade III in intensity,2. A

5、bnormal S2,3.Abnormal ECG,4.Abnormal X-ray,5.Abnormal BP,TOF,PDA,VSD,ASD,Four Congenital Cardiac Anomalies in Children,返回,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 adul

6、t 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.,Congenital Cardiac Anomalies in Children,Atrial Septal Defect Three types of atrial septal defect are classified on an

7、 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.,房间隔缺损,房间隔缺损原发孔型,位于房间隔的下部，紧邻房室瓣。,房间隔缺损继发孔型,亦称中央型或卵圆孔型,房间隔缺损静脉窦型,位置接近上腔静脉,房间隔缺损,Congenital Cardiac Anomali

8、es in Children,Atrial Septal Defect Blood is chronically 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

9、抗 in the pulmonary arterial system, causing a wide 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 audibl

10、y split during expiration. A chest x-ray usually 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.,Congenital Card

11、iac Anomalies in Children,Atrial 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 mos

12、t severe cases, an irreversible plexiform arteriopathy丛状的动脉病, 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

13、 becomes bidirectional, and finally reverses; a right ventricular pressure 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 overlo

14、ad; it usually affects 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 dif

15、ficult to differentiate clinically between ASD and such conditions as cardiomyopathy 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 patie

16、nt in whom 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.,Congenital Cardiac Anomalies in Children,Atrial Septal Defect Surgery is contraindicated 治疗或处置不当 whe

17、n 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.,Congenital Cardiac Anomalies in Children,Atrial Septal Defect Nonsurgical closure us

18、ing 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.,房间隔缺损封堵伞,房间隔缺损封堵过程,Congenital Cardiac Anomalies in Children,Ventricular Septal Defect (VSD) VSD is the most common cong

19、enital 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 high; closure occurs particularly often in infancy but also in later y

20、ears.,VSD,VSD,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 pulmonary a

21、rtery.,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 failure symptoms and t

22、he maintenance of normal growth.,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. Infants between 6 and 12 mo of ag

23、e with large defects associated with pulmonary hypertension, 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 est

24、ablished, but surgery appears to be highly effective as a prophylactic measure.,室间隔缺损封堵伞,室间隔缺损封堵,Patent Ductus Arteriosus,PDA 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

25、 lesions and can often play a critical role in some lesions. Female : Male ratio of 2:1 Often associated w/ coarctation & VSD.,动脉导管未闭PDA,Patent Ductus Arteriosus,Question: What TORCH infection is PDA associated with? Answer: Rubella,Patent Ductus Arteriosus,Hemodynamics As a result of higher aortic

26、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.,Patent Ductus Arteriosus,Hemodynamics Large 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.,Patent Ductus Arteriosus,Clinical Signs & Sy