1. الدكتور بشار عبد الوهيب الحاج علي استشاري بطب الأطفال والرضع وحديثي الولادة رئيس دائرة المشافي والهيئات العامة - وزارة الصحة

2. ما هو المقصود بآفات القلب الولادية أو الخلقية

3. ما هو سبب حدوث آفة القلب الولادية السبب الحقيقي غير معروف عوامل وراثية بدئية ( شذوذات صبغية - عوامل جينية ) عوامل بيئية ( كيمائية – أدوية – فيروسية – اشعة – مرض عند الأم ) عوامل متداخلة ( متعددة العوامل )

4. Classification A cyanotic Interrupted Aortic Arch Aortic Stenosis Ventricular Septal Defect Atrial Septal Defect Cyanotic Defects Hypo plastic left Ventricle Pulmonary Stenos is Tetra logy of Fallout D transposition of the great vessels Tricuspid Atresia

5. A cyanotic Congenital heart Disease

6. Ventricular Septal Defect Defect in the septum separating the left and right ventricles Most common type of congenital heart disease accounting for 21% of all cases Can occur singly or in multiples anywhere along the ventricular septum Small defects often close spontaneously in the first 2 years of life while large defects require surgical repair within the 1 st year

7. Ventricular Septal Defect

8. Symptoms of Ventricular Septal Defects Rapid breathing Irritability Excessive Sweating Poor weight gain Congestive Heart Failure, usually within 6 to 8 weeks of life if defect is large Pulmonary Hypertension if defect is large

9. Treatments for Ventricular Septal Defects Lasix and Aldactone to decrease symptoms of CHF Digoxin to increase effectiveness of myocardial function If surgery needed, patching or suturing the defect can be done Mortality from surgery is low

10. Atrial Septal Defect Defect in the septum separating the left and right atria Accounts for 5-10% of congenital heart disease Twice as frequent in girls versus boys Three types of atrial septal defects

11. Atrial Septal Defect Ostium Primum: Defect located in the lower part of septum near tricuspid valve which separates the right atrium and right ventricle Ostium Secundum: Defect located near center of atria septum (most common accounting for 50-70% of atrial defect) Sinus Venosus: Located near the SVC or IVC’s entrances to the heart

12. Atrial Septal Defect

13. Symptoms of Atrial Septal Defects Slender build Heart murmur resulting from increased blood flow through pulmonary valve Usually no significant exercise restriction unless defect is large. SOB or palpitations are possible.

14. Treatment of Atrial Septal Defects If defect is small (less than 2mm), will usually resolves spontaneously If defect is large, surgical correction is needed Minimally invasive procedures available Transcatheter devices, such as a septal occluder may be used.

15. Patent Ductus Arteriosus The ductus arteriosus connects the pulmonary artery to the descending aorta during fetal life. PDA results when the ductus fails to close after birth. Picture: www.lpch.org

16. Patent Ductus Arteriosus Pathophysiology: Blood flows from aorta to the pulmonary artery, creating a left to right shunt, resulting in left atrium and ventricle overload. Increased pulmonary blood flow can result in pulmonary hypertension and reversal of the shunt, which is known as Eisenmenger’s Syndrome. This results in flow of desaturated blood to the lower extremities. Picture: www.lpch.org

17. Patent Ductus Arteriosus Symptoms: Children with small patent ductus are usually asymptomatic. Large left to right shunts develop symptoms of congestive heart failure such as tachypnea, tachycardia, poor feeding and slow growth Physical exam: Continuous murmur heard best at the left sternal border.

18. Patent Ductus Arteriosus Lab Studies: CXR: enlarged cardiac silhouette secondary to left atrial and ventricular enlargement with prominent pulmonary vascular markings. EKG: left atrial enlargement, LVH ECHO: doppler flow through the ductus Treatment: Surgical division or ligation of the PDA

19. Interrupted Aortic Arch Relatively rare occurs in 2 cases per 100,000 live births Consists of 2 different defects: divided aortic arch and ventricular septal defect Divided Aortic arch results in continued blood flow to the upper extremities, but none to the lower extremities

20. Interrupted Aortic Arch Lower extremities oxygenated due to the combination of patent ductus arteriosus and ventricular septal defect PDA connects lower portion of the aortic to the pulmonary artery VSD allows travel of oxygenated blood to the right ventricle which in turns travels to the pulmonary artery.

21. Interrupted Aortic Arch

22. Symptoms of Interrupted Aortic Arch Often discovered 3-4 days after birth when the patent ductus arteriosus closes Symptoms of shock develops very rapidly as no oxygenated blood flows to the lower extremities Rapid breathing, clammy sweating, and poor feeding often develops during the first week

23. Symptoms of Interrupted Aortic Arch Most babies born at term with normal length and weight Heart murmur usually heard Liver may be enlarged Left arm/leg pulses may be diminished or absent Echocardiogram for diagnosis

24. Treatment of Interrupted Aortic Arch Before surgery, try to keep the PDA open to provide oxygenated blood to the lower extremities Surgery to suture together the two ends of the aorta, patch the VSD, and legate the PDA 85-90% patients survive the hospital stay

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26. Cyanotic Heart Diseases

27. TETRALOGY OF FALLOT It is the most common cyanotic congenital heart defect VSD, pulmonary stenosis, overriding aorta and right ventricular hypertrophy.

29. Clinical Manifestations The degree of cyanosis depends on the amount of pulmonary stenosis. Infants initially may be acyanotic. A pulmonary stenosis murmur is the usual initial abnormal finding. If the pulmonary stenosis is more severe, or as it becomes more severe over time, the amount of right-to-left shunting at the VSD increases, and the patient becomes more cyanotic. With increasing severity of pulmonary stenosis, the murmur becomes shorter and softer. In addition to varying degrees of cyanosis and a murmur, a single S 2 and right ventricular impulse at the left sternal border are typical findings.

30. Imaging Studies The ECG usually has right axis deviation and right ventricular hypertrophy.  The classic chest x-ray finding is a boot-shaped heart created by the small main pulmonary artery and upturned apex secondary to right ventricular hypertrophy.  Echocardiography shows the anatomic features, including the levels of pulmonary stenosis, and provides quantification of the degree of stenosis.  Coronary anomalies, specifically a left coronary artery crossing the anterior surface of the right ventricular outflow tract, are present in 5% of patients with tetralogy of Fallot.

31. Treatment Treatment of hypoxic spells consists of oxygen administration, placing the child in the knee-chest position (to increase venous return), and giving morphine sulfate (to relax the pulmonary infundibulum and for sedation). Complete surgical repair with closure of the VSD and removal or patching of the pulmonary stenosis can be performed in infancy. Occasionally, palliative shunt surgery between the subclavian artery and pulmonary artery is performed for complex forms of tetralogy of Fallot with more complete repair at a later time. Subacute bacterial endocarditis prophylaxis is indicated

32. TRANSPOSITION OF THE GREAT ARTERIES Although dextroposed transposition of the great arteries represents only about 5% of congenital heart defects it is the most common cyanotic lesion to present in the newborn period Without mixing of the two circulations, death occurs quickly. Mixing can occur at the atrial (patent foramen ovale/ASD), ventricular (VSD), or great vessel (PDA) level.

34. Clinical Manifestations A history of cyanosis is always present, although it may not be profound and depends on the amount of mixing. Quiet tachypnea and a single S 2 are typically present. If the ventricular septum is intact, there may be no murmur. Children with transposition and a large VSD have improved intra cardiac mixing. They may present with signs of CHF. The heart is hyperdynamic, with palpable left and right ventricular impulses. A loud VSD murmur is heard. S 2 is single.

35. Imaging Studies ECG findings typically include right axis deviation and right ventricular hypertrophy. The chest x-ray reveals increased pulmonary vascularity, and the cardiac shadow is classically an egg on a string created by the narrow superior mediastinum. Echocardiography shows the transposition of the great arteries, the sites and amount of mixing, and any associated lesions

36. Treatment Initial medical management includes prostaglandin E 1 to maintain ductal patency. If the infant remains significantly hypoxic on prostaglandin therapy, a balloon atrial septostomy is performed to improve mixing between the two circulations. Complete surgical repair is most often an arterial switch; the atrial switch is rarely done. The arterial switch usually is performed within the first 2 weeks of life, when the left ventricle still can maintain a systemic pressure.

37. TRICUSPID ATRESIA Tricuspid atresia accounts for approximately 2% of all congenital heart defects. The absence of the tricuspid valve results in a hypoplastic right ventricle. All systemic venous return must cross the atrial septum into the left atrium. The presence of a PDA or VSD also is necessary for pulmonary blood flow and survival.

39. Clinical Manifestations Infants with tricuspid atresia are usually severely cyanotic and have a single S 2. If a VSD is present, there may be a murmur. A diastolic murmur across the mitral valve may be audible. Frequently there is no significant murmur.

40. Imaging Studies The ECG shows left ventricular hypertrophy and a superior QRS axis (between 0 and -90 degrees). The chest x-ray reveals a normal or mildly enlarged cardiac silhouette with decreased pulmonary blood flow. Echocardiography shows the anatomy, associated lesions, and source of pulmonary blood flow.

41. Treatment Management initially depends on the presence of a VSD and the amount of blood flow across the VSD to the lungs. If there is no VSD, or it is small, prostaglandin E 1 is used to maintain pulmonary blood flow until surgery. Surgery is staged with an initial subclavian artery-to- pulmonary shunt (Blalock-Taussig procedure) typically followed by a two-stage procedure (bidirectional Glenn and Fontan procedure), which directs systemic venous return directly to the pulmonary arteries

42. TRUNCUS ARTERIOSUS Truncus arteriosus occurs in less than 1% of all cases of congenital heart disease. It results from the failure of septation of the truncus, which normally occurs during the first 3 to 4 weeks of gestation. Anatomically a single arterial trunk arises from the heart with a large VSD immediately below the truncal valve. The pulmonary arteries arise from the single arterial trunk either as a single vessel that divides or individually from the arterial trunk to the lungs.

44. Clinical Manifestations Varying degrees of cyanosis depend on the amount of pulmonary blood flow. If not diagnosed at birth, the infant may develop signs of CHF as pulmonary vascular resistance decreases. The signs include tachypnea and cough. Peripheral pulses are usually bounding as a result of the diastolic runoff into the pulmonary arteries. A single S 2 is due to the single valve. There may be a systolic ejection click, and there is often a systolic murmur at the left sternal border.

45. Imaging Studies ECG findings include combined ventricular hypertrophy and cardiomegaly. Increased pulmonary blood flow is usually seen on chest x-ray. Pulmonary arteries may appear displaced. Echocardiography defines the anatomy, including the VSD, truncal valve function, and origin of the pulmonary arteries.

46. Treatment  Medical management is usually needed and includes anticongestive medications.  Surgical repair includes VSD closure and placement of a conduit between the right ventricle and pulmonary arteries

47. TOTAL ANOMALOUS PULMONARY VENOUS RETURN Total anomalous pulmonary venous return accounts for about 1% of congenital heart disease. Disruption of the development of normal pulmonary venous drainage during the third week of gestation results in one of four abnormalities. Anatomically, all of the pulmonary veins fail to connect to the left atrium and return abnormally via the right heart. They may have supracardiac, cardiac, infracardiac, or mixed drainage. An atrial level communication is necessary for systemic cardiac output and survival

49. Clinical Manifestations The most important determinant of presentation is the presence or absence of obstruction to the pulmonary venous drainage. Infants without obstruction have minimal cyanosis and may be asymptomatic. The pulmonary blood flow creates a continuous murmur and reenters the right atrium and right ventricle. There is a hyperactive right ventricular impulse with a widely split S 2 (owing to increased volume ejected from the right ventricle) and a systolic ejection murmur at the left upper sternal border. Growth is relatively poor. Infants with obstruction present with cyanosis, marked tachypnea and dyspnea, and signs of right heart failure including hepatomegaly. The obstruction results in little, if any, increase in right ventricular volume. There may be no murmur or changes in S 2

50. Imaging Studies For infants without obstruction, the ECG is consistent with right ventricular volume overload. Cardiomegaly with increased pulmonary blood flow is seen on chest x-ray. Infants with obstructed veins have right axis deviation and right ventricular hypertrophy on ECG. On chest x- ray, the heart is normal or mildly enlarged with varying degrees of pulmonary edema that can appear similar to hyaline membrane disease or pneumonia. Echocardiography shows the volume-overloaded right heart, right-to-left atrial level shunting, and common pulmonary vein including site of drainage and degree of obstruction

51. Treatment At surgery, the common pulmonary vein is opened into the left atrium, and there is ligation of any vein or channel that had been draining the common vein.

52. HYPOPLASTIC LEFT HEART SYNDROME Hypoplastic left heart syndrome accounts for 1% of all congenital heart defects. It is the most common cause of death from cardiac defects in the first month of life. Hypoplastic left heart syndrome occurs when there is failure of development of the mitral or aortic valve or the aortic arch. A small left ventricle that is unable to support normal systemic circulation is a central finding of hypoplastic left heart syndrome, regardless of etiology. Associated degrees of hypoplasia of the ascending aorta and aortic arch are present. Left-to-right shunting occurs at the atrial level

54. Clinical Manifestations After delivery, the infant is dependent on right-to-left shunting at the ductus arteriosus for systemic blood flow. As the ductus arteriosus constricts, the infant becomes critically ill with signs and symptoms of CHF from excessive pulmonary blood flow and obstruction of pulmonary venous return. Pulses are diffusely weak or absent. S 2 is single and loud. There is usually no heart murmur. Cyanosis may be minimal, but low cardiac output gives a grayish color to the cool, mottled skin.

55. Imaging Studies ECG findings include right ventricular hypertrophy with decreased left ventricular forces. The chest x-ray reveals cardiomegaly (with right- sided enlargement) and pulmonary venous congestion or pulmonary edema. Echocardiography shows the small left heart, the degree of stenosis of the aortic and mitral valves, the hypoplastic ascending aorta, and the adequacy of left- to-right atrial flow.

56. Treatment Medical management includes prostaglandin E 1 to open the ductus arteriosus, correction of acidosis, and ventilatory and blood pressure support as needed. Surgical repair is staged with the first surgery (Norwood procedure) done in the newborn period. Subsequent procedures create a systemic source for the pulmonary circulation (bidirectional Glenn and Fontan procedures), leaving the right ventricle to supply systemic circulation. There have been and continue to be many modifications to all three stages of the surgical repair.

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