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CONGENITAL HEART DISEASES

CONGENITAL HEART DISEASES. ACYANOTIC HEART DISEASE. Acyanotic Heart Diseases. LEFT TO RIGHT SHUNTS. OBSTRUCTIVE LESIONS. REGURGITANT LESIONS. LEFT TO RIGHT SHUNT. Determinants of L to R shunting. Size Of the Defect. Relative Compliance of the Right and Left Ventricle.

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CONGENITAL HEART DISEASES

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  1. CONGENITAL HEART DISEASES

  2. ACYANOTIC HEART DISEASE

  3. Acyanotic Heart Diseases LEFT TO RIGHT SHUNTS OBSTRUCTIVE LESIONS REGURGITANT LESIONS

  4. LEFT TO RIGHT SHUNT

  5. Determinants of L to R shunting Size Of the Defect Relative Compliance of the Right and Left Ventricle Relative Vascular Resistance in the Pulmomary and Systemic Circulations

  6. LEFT TO RIGHT SHUNT Communication between the pulmonic and systemic circulation Increase blood flow to the lungs HEART FAILURE SIGNS

  7. Heart Remodeling (dilatation of the heart) Increase Sympathetic nervous system Increase pulmonary vascular resistance (EISENMENGER PHYSIOLOGY)

  8. Left to Right Shunt • Atrial Septal Defect • Atrioventricular Septal Defect • Partial Anomalous Pulmonary Venous Retur • Ventricular Septal Defect • PDA • Coronary-AV Fistula • Ruptured Sinus of Valsalva Aneurysm

  9. Atrial Septal Defect

  10. Atrial Septal Defect • Secundum • Most common form and is associated with structurally normal atrioventricular valves • Region of the fossa ovalis • May be single or fenestrated, openings ≥2cm in largest diameter are common in symptomatic older children

  11. Atrial Septal Defect Enlargement of the pulmonary artery Enlargement of the right atrium Normal Left Ventricle and Aorta

  12. Atrial Septal Defect • Clinical Manifestations and findings • Wide and fixed splitting 2nd heart sound • Mild left precordial bulge • Right ventricular systolic lift on the left sternal border • Systolic ejection murmur at the left middle and upper sternal border • Loud 1st heart sound and sometimes a pulmonic ejection click • Short rumbling Mid-diastolic murmur on the lower left sternal border

  13. Atrial Septal Defect • Diagnostics • Chest Xray:RVE, RAE, pulmonary artery is large and pulmonary vascularity is increased • ECG: normal or right axis deviation and a minor right ventricular conduction delay (rsR pattern in the right precordial leads

  14. Atrial Septal Defect • Diagnostics • 2D echo • increased right ventricular-end-diastoic dimension and flattening and abnormal motion of the ventricular septum (anterior movement in systole or it remains straight. • ASD confirmed by pulsed and color flow Doppler

  15. Atrial Septal Defect • Treatment • Surgical or transcatheter device closure is advised for all symptomatic patients and also for asymptomatic patients with a Qp:Qs ratio of at least 2:1

  16. Sinus Venosus Atrial Septal Defect • Upper part of the atrial septum in close relation to the entry of the SVC • May be related to Partial Anomalous Pulmonary Venous return • Sometimes the superior vena cava straddles the defect (rarely involves the IVC) • Tx: Surgical

  17. Partial Anomalous Pulmonary Venous Return • May drain into the: SVC or IVC, Right atrium, Coronary Sinus • May involve some or all of the veins from only 1 lung (right>left) • Scimitar Syndrome • An anomalous vein draining into the IVC is visible on Chest Xray as a crescentic shadow of vascular density along the right border of the cardiac silhouette

  18. Scimitar Syndrome

  19. Partial Anomalous Pulmonary Venous Return • Dx: 2D Echo • Cardiac catheterization • Selective pulmonary arteriography: presence of anomalous pulmonary veins • Descending aortography: anomalous pulmonary arterial supply to the right lung • Prognosis: Excellent • Tx: Surgical if large left-to-right shunting

  20. Atrioventricular Septal Defects(Ostium Primum and AV Canal Defects) • Situated in the lower portion of the atrial septum and overlies the mitral and tricuspid valve • A cleft in the anterior leaflet of the mitral valve can be seen

  21. Atrioventricular Septal Defects(Ostium Primum) • Pathophysiology: left to right shunt across the atrial defect and mitral (or occasionally tricuspid insufficiency • Pulmonary arterial pressure is typically normal or only mildly increased

  22. Atrioventricular Septal Defects(Ostium Primum) • Clinical Manifestations • Asymptomatic • History of exercise intolerance, easy fatigability and recurrent pneumonia (with large defects and severe mitral insufficiency) • Harsh or occasionally high-pitched apical holosystolic murmur (due to mitral insufficiency) • Cardiac enlargement and hyperdynamic precordium

  23. Atrioventricular Septal Defects(Ostium Primum) • Clinical Manifestations • Other findings: normal or accentuated 1st heart sound; wide, fixed splitting of the 2nd sound; pulmonary ejection murmur sometimes preceeded by a click; low-pitched and diastolic rumbling murmur at te lower left sternal edge or apex or both

  24. Atrioventricular Septal Defects(AV Canal Defects) • AV canal defect or endocardial cushion defect • Contiguous AV septal defects with markedly abnormal AV valves

  25. Atrioventricular Septal Defects(Ostium Primum and AV Canal Defects) • Complete form: single AV valve common to both ventricles and consists of an anterior and a posterior bridging leaflet related to the ventricular septum with a lateral leaflet in each ventricle • Common toDown syndrome

  26. Atrioventricular Septal Defects(AV Canal Defects) • Left or right dominant AVSD due to hypoplasia of one of the ventricles

  27. Atrioventricular Septal Defects(AV Canal Defect) • Pathophysiology • L to R shunting occurs at both atrial and ventricular levels; with shunting from the left ventricle to the right atrium (due to the absence of the AV septum) • Pulmonary hypertension and increased tendency to develop pulmonary vascular resistance  right to left shunting  cyanosis (Eisenmenger syndrome) • AV valvular insufficiency

  28. Atrioventricular Septal Defects(AV Canal Defect) • Clinical Manifestations • Heart failure and intercurrent pulmonary infection • Enlarged liver • Failure to thrive

  29. Atrioventricular Septal Defects(AV Canal Defect) • PE findings • Cardiac enlargement • Systolic thrill at the lower left sternal border • Precordial bulge and lift • Normal or accentuated 1st heart sound • Widely split 2nd heart sound • Low pitched, mid-diastolic rumbling murmur, lower left sternal border • Pulmonary systolic ejection murmur • Harsh apical holosystolic murmur

  30. Atrioventricular Septal Defects(AV Canal Defect) • CXR: prominent ventricles and atrium • 2D echo • RVE with encroachment of the mitral valve echo on the left ventricular outflow tract • “gooseneck” deformity of the left ventricular outflow tract • Both valves insert at the same level • Common AV valve

  31. Atrioventricular Septal Defects(AV Canal Defect) • Treatment • Ostium septum defect: Patch prosthesis for the closure of ASD and direct suture for the cleft in the mitral valve • AV septum defect: surgical operation during infancy (due to the risk of pulmonary vascular disease as early as 6-12 months of age)

  32. Patent foramen Ovale • Not an ASD • Left to right shunting is unusual but may occur in the presence of a large volume load or hypertensive left atrium • Does not require surgical treatment but may be a risk for paradoxical systemic embolization. • Device closure is considered if there is a history of thromboembolic stroke

  33. Ventricular Septal Defect • Most common cardiac malformation (25% of CHD) • Different types: • Membranous type: most common • Infundibular types • Muscular • Supracrista

  34. Ventricular Septal Defect • Determinants of the magnitude of the L to R shunting • Size • restrictive VSD (<5mm) • Level of Pulmonary resistance in relation to systemic resistance • Nonrestrictive VSD (>10mm)

  35. Ventricular Septal Defect • At birth, the pulmonary vascular resistance is elevated, thus the size of the left to right shunting is limited • Few weeks after birth, there is decrease in pulmonary resistance • With continued exposure of the pulmonary vascular bed to high systolic pressure and high flow, pulmonary vascular obstructive disease develops  Eisenmenger syndrome

  36. Ventricular Septal Defect • Clinical Manifestations • Small VSDs • Asymptomatic • Loud, harsh or blowing holosystolic murmur on the left lower sternal border (frequently with thrill) • In neonates with VSD on the apical muscular septum, murmur heard on the apex

  37. Ventricular Septal Defect • Clinical Manifestations • Large VSD • Dyspnea, feeding difficulties, poor growth, profuse perspiration, recurrent pulmonary infections and cardiac failure • Cyanosis • Prominence of the left precordium (palpable lift) • Laterally displaced apical impulse and apical thrust • Systolic thrill

  38. Ventricular Septal Defect • Clinical Manifestations • Large VSD • Less harsh but more blowing systolic murmur • Increased pulmonic component of the 2nd heart sound • Mid-diastolic, Low pitched rumble at the apex • Increased blood flow to the mitral valve • Qp:Qs ratio ≥2:1

  39. Ventricular Septal Defect • Diagnosis • Chest Xray • Small VSDs: normal or minimal cardiomegaly and borderline increased in pulmonary vasculature • Large VSDs • Gross cardiomegaly with prominence of both ventricles, left atrium and pulmonary artery • Pulmonary vascular markings are increased • Pulmonary edema

  40. Ventricular Septal Defect • Clinical Manifestations • 2D echo • Helpful in estimating shunt size, the degree of volume overload, the increased dimensions of chambers and presence of other valve defects (including aortic valve insufficiency or prolapse) • Pulse doppler examination calculates pressure gradient across the defect

  41. Ventricular Septal Defect • Treatment • 30-50% close spontaneously, most frequently during the 1st 2 years of life • Small muscular VSDs > membranous

  42. Ventricular Septal Defect • Treatment • Indications for surgical closure • Large defects in whom clinical symptoms and failure to thrive cannot be controlled medically • Infants between 6 and 12 months with large defects associated with pulmonary hypertension • Patients older than 24 months with Qp:Qs ratio greater than 2:1 • Supracristal VSD • Contraindication to surgery: severe pulmonary vascular disease nonresponsive to pulmonary vasodilators

  43. Patent Ductus Arteriosus • Location: the aortic end is distal to the origin of the left subclavian artery and enters the pulmonary artery at its bifurcation • Associated with maternal rubella infection • Preterm: the smooth muscle in the wall is less responsive to high PO2 and less likely to constrict after birth; normal structure PDA • Term: the wall is deficient in both the mucoid endothelial layer and the muscular media

  44. Patent Ductus Arteriosus • Clinical Manifestations • Bounding peripheral pulses and a wide pulse pressure, due to runoff of blood into the pulmonary artery during diastole • Machinery like murmur • Thrill, maximal in the 2nd left interspace with radiation toward the left clavicle, left sternal border and apex

  45. Patent Ductus Arteriosus • Treatment • Surgical or catheter closure

  46. Aorticopulmonary Window Defect • Consists of a communication between the ascending aorta and the main pulmonary artery • Unlike truncus arteriosus, there is presence of pulmonary and aortic valves and an intact ventricular septum • Systolic murmur with an apical mid-diastolic rumble (due to increased blood flow across the mitral valve) • Tx: surgical

  47. Coronary-Cameral Fistula • A congenital fistula existing between a coronary artery and an atrium, ventricle or pulmonary artery • Clinical signs may be similar to PDA but diffuse • Diagnosis: doppler echocardiography and cardiac catheterization • Treatment: small fistuals may close spontaneously, larger fistulas may require catheter intervention or surgical closure of the fistula

  48. Ruptured Sinus of Valsalva Aneurysm • Happens when one of the valsalva of the aorta is weakened by congenital or acquired disease and ruptures in to the right atrium or ventricle • Acute heart failure with new loud to and fro murmur • Left to right shunt at the area of the atrium or ventricle • Urgent surgical repair is required

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