CONGENITAL HEART DISEASE

1. CONGENITAL HEART DISEASE 1- Zahra Asghari 2- Samira Baniasadimoghadam 3-Mahtab Dolatabadi 4- FahimeGohari 5-Aida Mirkazemi 6- AzadeIzadi 7- SanazkhosraviGhareche

2. approximately 0.8% of all live births are complicated by congenital cardiac abnormalities. • Congenital heart disease is a major cause of • infant morbidity and mortality. • As a result of advances in pediatric cardiology and cardiothoracic surgery, approximately 85% of infants born with congenital heart diseasecan be expected to survive into adulthood. • Most cases of congenital heart disease occur sporadically.

3. Etiology • without a known specific cause. • Genetic abnormalities are responsible for a proportion of cases and may contribute to cases occurring sporadically as well • Environmental factors. • Symptoms can develop shortly after birth when transition from fetal to adult circulation represents a new dependence on biventricular circulation with a pulmonary circuit.→begin of symptoms

4. The isolated pulmonary and systemic circulations: • ①D: the ductusarteriosus and the foramen ovale. • ②L :Hypertrophy of the morphologic right ventricle in L transposition of the great arteries is sufficient to compensate for systemic vascular resistance and maintain normal perfusion for years with symptoms often developing when the systemic ventricle fails.

5. Still other lesions may develop in adulthood when degenerative changes, such as stenosis of apreviously well-functioning bicuspid aortic valve, are superimposed on an initial lesion. • congenital defects may : ★Go undetected throughout life →small ASDs ★resolve spontaneously →small muscular VSDs

6. Septal defects

7. ATRIAL SEPTAI DEFECTS(ASD) • ASDs are some of the most common congenital defects, representing 10%to l7% • a higher prevalence in women (6o%) • Defects are classified according to their location in the interatrial septum : • 1- primary ostium defect : 20%, defect in atrioventricular junction • usually, associated with a cleft mitral valve and mitral failure •  rarely, great ASD, single VA valve→defect in VA septum 2-secundary ostium defect:60%,defect in fossaovalis

8. uncomplicated ASDs →blood shunts hom the left to the right atrium • The magnitude of the shunting is determined by the size of the defect and the compliance of the Ieft and right ventricles. • Small ASDs :the increased blood flow in the right atrium without sequelae and no significant hemodynamic compromise of the right heart. • Large ASDs: the right atrium and right ventricle dilate to accommodate the increased volume of shunted blood. Pressurein the pulmonary artery increases econdary to the increased volume of blood.

9. Symptoms • Most patients with ASD are asymptomatic until adult. • when symptoms such as fatigue, dyspnea, and poor exercise tolerance develop, secondary to right ventricular dysfunction. • atrial fibrillation, especially after 50 years of age • 5%Irreversible pulmonary vascular obstruction resulting in right-to-left shunting and cyanosis (Eisenmenger syndrome) • Stroke or transient ischemic attack in order to paradoxical emboli

10. Physical exam • Parasternal RV impulse • Widely and fixed split S2 • Ejection murmur across pulmonic

11. Diagnosis • Two dimensional and color Doppler echocardiography • Transesophagial echocardiography • Electerocardiography • MRI • Cardiac catheterization • Angiography

12. Treatment • closure, even in the absence of symptoms, pulmonary hypertension,… • Significant pulmonary hypertension is a contraindication to ASD closure. • devise Amplatzer • Primum and sinus venosus defects should be surgically closed. • Antibiotic prophylaxis for infective endocarditis is not required for small ASDs or patent foramen ovale or after ASD closure.

13. Ventricular septal defect (VSD) • VSD is a common congenital abnormality in newborns.(1 in 500 births)

14. VSD’s classification • Supracristalvsd • Perimembranousvsd(most common:80%) • Atrioventricular canal vsd • Muscular vsd

15. If the defect is small: Right ventricular size & function are normal and pulmonary vascular resistance dose not increase. • If the defect is large: The right ventricle dilates to accommodate the increased volume & pulmonary blood flow increases.

16. syndromeEisenmenger • Pulmonary vascular obstruction • Pulmonary artery HTN • Reversal of interventricular shunt • Systemic desaturation • Cyanosis • clubbing

17. Physical examination • Hyperdynamicprecordium • Palpable thrill along the left sternal border • Holosystolic murmur • Loud p2

18. Diagnosis • Tow dimensional echocardiography • Doppler echocardiography • MRI • CT scan

19. Treatment: • Sutures • Prosthetic patch • Percutaneous device closure(muscular)

20. :Congenital AS & bicuspid aortic valve • Valvalrstenosis is most often secondary to a bicuspid aortiv valve. • Congenital left ventricular outflow obstruction occurs at valvular,subvalvularor supravalvular.

21. Bicuspid aortic stenosis: as with acquired aortic stenosis: chest pain,syncope,congestive heart failure. Other complications: sudden death & infective endocaditis.

22. Physical examination: • Ejection quality murmur at the left sternal border. • Early systolic ejection click • Decrescendo diastolic murmur of aortic insufficiency

23. Diagnosis • Two –dimensional echocardiography • Doppler echocardiography

24. Treatment: • Percutaneousvalvuloplasty • Aortic valve replacement • Antibiotic prophylaxis • Oral hygiene

25. Sub aortic stenosis: • Firs diagnosed in adulthood • A discrete,fibrousdiaghragm

26. Supravalvar aortic stenosis(svsa): • Loss-of-function mutations in the extracellular matrix protein,elastin,is responsible. • It is often part of a syndrome with hypercalcemia,multiple skeletal-vascular-developemental abnormalities.

27. PULMONIC VALVE STENOSIS

29. Pulmonic valve stenosis is the most common cause of obstruction to right ventricular outflow • usually occurs as an isolated congenital lesion

30. Fusion of the pulmonary leaflets creates the pressure-overloaded state and results in right ventricular hypertrophy • Unless the valve is severely stenotic at birth, most affected persons live a normal life until adolescence or young adulthood • Patients with mild-to-moderate stenosis are usually asymptomatic and rarely have complications associated with the defect • Patients with moderate-to-sever obstruction often exhibit progressive fatigue and dyspnea • If right ventricular dysfunction occurs, then symptoms and signs of right-sided ventricular heart failure may be present.

31. Physical examination • The patient with severe stenosis has a right ventricular lift on palpation of the precordium • The S I sound is usually normal and is followed by an opening click that becomes louder with expiration • The P2 sound becomes softer and is delayed as the severity of the stenosis increase • The characteristic murmur of pulmonicstenosis is a systolic ejection murmur heard best at the left upper sternal border, which increases with inspiration • A late-peaking murmur indicates more severe stenosis • A prominent jugular venous a wave and right-sided 54 sound may also be present in patients with severe obstruction to right ventricular outflow

32. treatment • For asymptomatic patients with mild pulmonicstenosis, therapy is limited to endocarditis prophylaxis • Patients with moderate stenosis (peak gradient >50mmHg) are likely to develop symptoms and require intervention over time and should be treated even in the absence of symptoms • Patients with severe obstruction (peak gradient >80mmHg) also require intervention • In children and adults with isolated pulmonicstenosis , percutaneous balloon valvuloplasty is a suitable therapeutic option • Valve replacement is rarely necessary

33. Ebstein's a nomaly • A rare condition (0.50loo f patients with congenital heart disease) characterized by apical displacement of the tricuspid valve into the right ventricle • The basal portion of the right ventricle forms part of the right atrium and leaves a small functional right ventricle • The tricuspid leaflets are often dysplastic and may partially adhere to the interventricular septum or right ventricular free wall • The degree of right ventricular dysfunction depends on the size of the functioning right ventricle and the severity of the tricuspid regurgitation • Frequently develops in adulthood • A patient foramen ovale or ostiumsecundum ASD is present in more than 50% of patients and may result in right-to-left shunt flow as right atrial pressure increases • Supraventricular arrhythmias are common

35. Diseases of Aorta • Coarctation of the aorta: • Coarrctation of the aorta is a firotic narrowing of the aortic lumen

36. Diagnosis • The diagnosis of coarctation may be made by tow-dimensional and Doppler echocardiography.

37. Repair in adults is recommended at at the time of diagnosis.Surgical repair is standard in many center,although primary percutaneous treatment with balloon angioplasty.

38. Patent DuctusArteriosus • A persistent communication between the aorta and pulmonary artery is the result of the failure of the ductusarteriosus to close.

39. Diagnosis And Treatment • Tow_dimensional and doppller • Percutaneous device closure • Surgery for the PDA that is too large

40. TETRALOGY OF FALLOT

41. DEFINITION • Tetralogy of fallot is the most common cyanotic congenital heart lesion in adults and represent 10% of all congenital heart defects

42. ETIOLOGY • Malalignment of the aorticopulmonary septum that divides the truncusarteriosus into the aorta and pulmonary artery during development.

43. FOUR COMPONENTS OF TETRALOGY • Overriding of the aorta in relation to the ventricular septum • Right ventricular outflow obstruction, which may be: valvular subvalvular supravalvular or combination of all threes • Membranous VSD • Right ventricular hypertrophy

46. The presence of right ventricular outflow obstruction is protective,preventing volume and pressure overload of the pulmonary circulation,which would result in fixed pulmonary hypertension. • If pulmonic stenosisi is mild, right - to - left shunt flow is minimal,and the patient remains acyanotic(Pink tetralogy) • The degree of right - to - left shunt flow depends on the degree of RV outflow obstruction.

47. Tetralogy may also be associated with ASD, muscular VSD, right aortic arch, and other coronary anomalies. • A chromosomal deletion (22q11) is found in 15% of cases, particulary in those whit associated anomalies.

48. TREATMENT • Surgical correction • Palliative surgery

49. Surgical correction • Usually performed during infancy or childhood • Involves relife of right ventricular obstruction and patch closure of the VSD

50. After reparative surgery • Residual polmunarystenosis or regurgitation • Aortic insuffiency • Residual VSD • Aneurysms of the right ventricular outflow tract • Sustained arrhythmias supraventricular ventricular