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1、Congenital Heart Disease,Jie Tian M.D. Childrens Hospital of CUMS,Epidemiology of CHD,Prevalence CHD occurs in 0.5-0.8% of live births; The incidence is higher among stillborns 死產(chǎn)(3-4%), abortuses流產(chǎn)(10-25%), and premature infants (about 2% excluding PDA); About 2-3 in 1,000 newborn infants will be s

2、ymptomatic with heart disease in the first year of life.,Epidemiology of CHD,Prevalence The diagnosis is established by 1 week of age in 40-50% of patients with CHD and by 1 month of age in 50-60% patients; With the advances in both palliative姑息and corrective surgery of the last 20 years, the number

3、 of children with CHD surviving to adulthood has increased dramatically;,Epidemiology of CHD,Prevalence Despite these advances, CHD remains the leading cause of death in children with congenital malformations; Most congenital defects are well tolerated in the fetus because of the parallel nature of

4、the fetal circulation.,Epidemiology of CHD,Etiology The cause of most CHD is unknown. Genetic factors play some role in CHD. About eight percent of cases result mainly from genetic factors. The heart defect is usually part of a chromosomal disorder such as Downs syndrome. However, no specific gene l

5、ocus for CHD has been identified.,Epidemiology of CHD,Etiology A child born to a parent with CHD has a substantially大體上increased likelihood of having a similar congenital lesion. The risk may be as high as 15 percent. For certain lesions, there appears to be a greater risk of transmission from the m

6、other than from the father.,Epidemiology of CHD,Etiology About two percent of cases of CHD are primarily the result of environmental or external factors. Such factors include rubella風(fēng)疹infection and ingestion of certain drugs, such as lithium鋰. CHD is a prominent component of the fetal alcohol syndro

7、me. The etiologic role of other agents, such as anticonvulsant medications and exogenous外源female sex hormones, is uncertain.,Noninvasive Diagnostic Studies,Chest X ray The location of the heart The size of the heart The relationship between heart and great vessel The blood flow of the lung,Noninvasi

8、ve Diagnostic Studies,Echocardiography Echocardiography has great value in assessing congenital cardiac anomalies and should usually be the first advanced diagnostic study to be carried out if the history, the physical examination, the chest X ray, and the electrocardiogram suggest the presence of c

9、ongenital heart disease.,Noninvasive Diagnostic Studies,Echocardiography The standard M-mode display and the two-dimensional display provide such information about cardiac anatomy as the size of the cardiac chambers, the connections of the great vessels, abnormalities of the valves, and subvalvular

10、obstructions.,Noninvasive Diagnostic Studies,Doppler ultrasonography Doppler ultrasonography is useful in detecting septal defects and directly assessing the amount of blood that shunts through the defect. The size of the shunt through a septal defect can also be estimated from Doppler ultrasound st

11、udies by comparing the velocity of the blood flow through the aorta with velocity through the pulmonary artery.,Noninvasive Diagnostic Studies,Transesophageal echocardiography Transesophageal echocardiography is particularly valuable for assessing atrial septal defects, but it also visualizes other

12、lesions effectively. Doppler studies are useful in assessing valvular stenosis and regurgitation as well.,Noninvasive Diagnostic Studies,Computed tomography (CT) CT provides a good display of the anatomic abnormalities associated with congenital heart disease and offers advantages over echocardiogra

13、phy in demonstrating anomalies involving the great vessels.,Computed tomography,Noninvasive Diagnostic Studies,Magnetic resonance imaging (MRI) MRI provides information similar to that provided by CT. MRI offers better resolution than CT without the disadvantages of the radiopaque不透射線的contrast mediu

14、m used in that technique.,Noninvasive Diagnostic Studies,Cardiac catheterization and selective angiocardiography are the most definitive diagnostic techniques currently available for use in congenital heart disease. However, noninvasive studies often provide information that is equivalent to that ob

15、tained from cardiac catheterization and is sufficient for planning surgical treatment.,Congenital Cardiac Anomalies 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 sur

16、gically 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.,Congenital Cardiac Anomalies in Children,Ventricular Septal Defect The VSD that do appear in adults as isol

17、ated 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,Ventricular Septal Defect In infants with a large VSD, medical management

18、 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 Anomalies in Children,Ventricular Septal Defect Indications for surgi

19、cal 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, even if symptoms are controlled by medication.,Congenital Ca

20、rdiac 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 prophylactic measure.,Congenital Cardiac Anomalies in Children,

21、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 physical signs are easily confused with the cardiac findings i

22、n 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 with a left-to-right shunt at the atrial level and volume

23、overwork of the right ventricle.,Congenital Cardiac Anomalies 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 pu

24、lmonary 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.,Congenital Cardiac Anomalies in Children,Atrial Septal Defect The splitting remains relatively fixed in relation to res

25、piration; 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 pulmonary trunk and increased pulmonary vascular markings. The relative severity of these conditions re

26、flects the size of the left-to-right shunt.,Congenital Cardiac 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

27、 after adolescence in about 15 percent of cases. In the most severe cases, an irreversible plexiform arteriopathy叢狀的動脈病, 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

28、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 the patient becomes cyanotic.,Congenital Cardiac Anomalies in Children,Atrial Septal Defect Right ventricular

29、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 or fibrillation and is often linked to tricuspid regurgitation. Eventually, a syndrome of right- and left-sided congesti

30、ve 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.,Congenital Cardiac Anomalies in Children,Atrial Septal Defect Surgical closure of ASD is a very safe and highly effective procedur

31、e. 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 ASD can be clinically diagnosed exhibit at least this degree of left-to-right shunt.,Congenital Cardiac Anomalies in Children,

32、Atrial Septal Defect Surgery is contraindicated 治療或處置不當(dāng) when 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 Anomal

33、ies in Children,Atrial Septal Defect Nonsurgical 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.,Congenital Cardiac Anomalies in Children,Tetralogy of Fallot

34、 The essential anatomic components of tetralogy of Fallot are ventricular septal defect and pulmonary stenosis. The degree of stenosis is usually severe enough to cause a predominantly right-to-left shunt, cyanosis, and diminished pulmonary blood flow. The pulmonary stenosis may be either valvular o

35、r infundibular; the infundibular form usually predominates in adults.,Congenital Cardiac Anomalies in Children,Tetralogy of Fallot The other two anatomic features present in tetralogy of Fallot are an overriding aorta and right ventricular hypertrophy. Variations exist in the degree of dextropositio

36、n of the aorta, but this anatomic defect rarely has functional importance.,Congenital Cardiac Anomalies in Children,Tetralogy of Fallot The technique of the totally corrective operation has been well established since the 1960s, and the defect is usually repaired in infancy or childhood. Patients wh

37、o survive to adulthood without an operation or with only a palliative shunt operation, in which a systemic artery is anastomosed匯合to the pulmonary artery, may display fairly good effort tolerance, show little or no cyanosis, and have a seemingly good prognosis.,Congenital Cardiac Anomalies in Childr

38、en,Tetralogy of Fallot Such patients, however, may become increasingly disabled as a result of gradual shunt closure and the progression of infundibular pulmonary stenosis. Furthermore, such complications as infective endocarditis, cerebral thromboembolism, and brain abscess may arise if the anomaly

39、 is not repaired .,Congenital Cardiac Anomalies in Children,Tetralogy of Fallot Total correction of TOF involves an operative mortality risk of less than five percent, and the late results are generally good, even though pulmonary valvular regurgitation and impaired contraction of the right ventricl

40、e occur in many patients. Corrective surgery should therefore be considered in any patient with TOF, regardless of whether a previous shunt operation has been performed.,Case report,One girl with 6 year old Symptoms: recurrent chest infections/wheeze Physical signs A fixed and widely split second heart sound An ejection systolic murmur best heard in the third left intercostal space,Chest X-ray showed right atrial and ventricular enlargement with increaded pulmonary vascular markings,What is this disease?,Atrial Septal Defect,