Congenital Heart Disease


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Congenital Heart Disease

  1. 1. Congenital Heart Disease Thoracic Conference Frank Nami, M.D.
  2. 2. The Heart
  3. 3. Congenial Heart Disease <ul><li>Obstructive Congenital Heart Lesions </li></ul><ul><li>Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow </li></ul><ul><li>Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow </li></ul>
  4. 4. Obstructive Congenital Heart Lesions <ul><li>Impede the forward flow of blood and increase ventricular afterloads. </li></ul><ul><li>Pulmonary Stenosis </li></ul><ul><li>Aortic Stenosis </li></ul><ul><li>Coarctation of the Aorta </li></ul>
  5. 5. Pulmonary Stenosis <ul><li>No symptoms in mild or moderately severe lesions. </li></ul><ul><li>Cyanosis and right-sided heart failure in patients with severe lesions. </li></ul><ul><li>High pitched systolic ejection murmur maximal in second left interspace. </li></ul><ul><li>Ejection click often present. </li></ul>
  6. 6. Pulmonary Stenosis
  7. 7. Aortic Stenosis <ul><li>Valvular Aortic Stenosis </li></ul><ul><li>Subaortic Stenosis </li></ul><ul><li>Supravalvular Aortic Stenosis </li></ul><ul><li>Asymmetric Septal Hypertrophy (Idiopathic Hypertrophic Subaortic Stenosis) </li></ul>
  8. 8. Valvular Aortic Stenosis <ul><li>Most common type, usually asymptomatic in children. </li></ul><ul><li>May cause severe heart failure in infants. </li></ul><ul><li>Prominent left ventricular impulse, narrow pulse pressure. </li></ul><ul><li>Harsh systolic murmur and thrill along left sternal border, systolic ejection click. </li></ul>
  9. 9. Valvular Aortic Stenosis <ul><li>Predominantly in males </li></ul><ul><li>Thickened, fibrotic, malformed aortic leaflets. </li></ul><ul><li>Fused commissures </li></ul><ul><li>Bicuspid aortic valve. </li></ul>
  10. 10. Valvular Aortic Stenosis
  11. 11. Coarctation of the Aorta <ul><li>Absent or weak femoral pulses. </li></ul><ul><li>Systolic pressure higher in upper extremities than in lower extremities; diastolic pressures are similar. </li></ul><ul><li>Harsh systolic murmur heard in the back. </li></ul>
  12. 12. Coarctation of the Aorta <ul><li>Males twice as frequently as females. </li></ul><ul><li>98% of all coarctations at segment of aorta adjacent to ductus arteriosus. </li></ul><ul><li>Produced by both an external narrowing and an intraluminal membrane. </li></ul><ul><li>Blood flow to the lower body maintained through collateral vessels. </li></ul>
  13. 13. Coarctation of the Aorta
  14. 14. Congenital Heart Lesions that INCREASE Pulmonary Arterial Blood Flow <ul><li>Atrial Septal Defect </li></ul><ul><li>Complete Atrioventricular Canal </li></ul><ul><li>Ventricular Septal Defect </li></ul><ul><li>Patent Ductus Arteriosis </li></ul><ul><li>Total Anomalous Pulmonary Venous Connection </li></ul><ul><li>Truncus Arteriosus </li></ul>
  15. 15. Atrial Septal Defect <ul><li>Acyanotic; asymptomatic, or dyspnea on exertion. </li></ul><ul><li>Right ventricular lift. </li></ul><ul><li>Fixed, widely split second heart sound. </li></ul>
  16. 16. Atrial Septal Defect <ul><li>Average life expectancy reduced because of right ventricular failure, dysrhythmias, and pulmonary vascular disease. </li></ul><ul><li>Surgical closure is recommended. </li></ul>
  17. 17. Atrial Septal Defect
  18. 18. Atrial Septal Defect
  19. 19. Atrial Septal Defect
  20. 20. Complete Atrioventricular Canal <ul><li>Heart failure common in infancy. </li></ul><ul><li>Cardiomegaly, blowing pansystolic murmur, other variable murmurs. </li></ul><ul><li>Deficiencies of both atrial and ventricular septal cushions and abnormalities of both mitral and tricuspid valves. </li></ul>
  21. 21. Complete Atrioventricular Canal <ul><li>Partial and complete AV canal defects frequently accompany Down’s syndrome. </li></ul><ul><li>Early surgical correction. </li></ul><ul><li>Reconstruction of the AV valves and closure of the septal defects by a single or double patch technique. </li></ul>
  22. 22. Complete Atrioventricular Canal
  23. 23. Complete Atrioventricular Canal
  24. 24. Ventricular Septal Defect <ul><li>Asymptomatic if defect is small. </li></ul><ul><li>Heart failure with dyspnea, frequent respiratory infections, and poor growth if defect is large. </li></ul><ul><li>Pansystolic murmur maximal at the left sternal border. </li></ul>
  25. 25. Ventricular Septal Defect <ul><li>Often one component of another more complex congenital heart lesion. </li></ul><ul><li>Heart is enlarged and lung fields are overcirculated. </li></ul><ul><li>Many of the defects will close spontaneously by age 7-8 years. </li></ul>
  26. 26. Ventricular Septal Defect
  27. 27. Ventricular Septal Defect
  28. 28. Patent Ductus Arteriosis <ul><li>Murmur usually systolic, sometimes continuous, “machinery” </li></ul><ul><li>Poor feeding, respiratory distress, and frequent respiratory infections in infants with heart failure. </li></ul><ul><li>Physical exam and echocardiography. </li></ul>
  29. 29. Patent Ductus Arteriosis <ul><li>Indomethacin, a prostaglandin E 1 inhibitor may close a PDA. </li></ul><ul><li>Surgical treatment after one week, by ligation, clipping, or division. </li></ul>
  30. 30. Patent Ductus Arteriosis
  31. 31. Patent Ductus Arteriosis
  32. 32. Total Anomalous Pulmonary Venous Connection <ul><li>Pulmonary veins do not make a direct connection with the left atrium. </li></ul><ul><li>Blood reaches the left atrium only through an atrial septal defect or patent foramen ovale. </li></ul><ul><li>Pulmonary congestion, tachypnea, cardiac failure, and variable cyanosis. </li></ul>
  33. 33. Total Anomalous Pulmonary Venous Connection <ul><li>Diagnosis by cardiac catherization or echocardiography. </li></ul><ul><li>Operative repair in all cases. </li></ul>
  34. 34. Truncus Arteriosus <ul><li>Single large vessel overrides the ventricular septum and distributes all the blood ejected from the heart. </li></ul><ul><li>Large VSD is present. </li></ul>
  35. 35. Truncus Arteriosus
  36. 36. Truncus Arteriosus <ul><li>Corrective operation with a valved conduit between right ventricle and pulmonary vessels. </li></ul><ul><li>Conduit will need to be changed as child grows but likelihood to develop pulmonary vascular disease is greatly reduced. </li></ul>
  37. 37. Congenital Heart Lesions that DECREASE Pulmonary Arterial Blood Flow <ul><li>Tetralogy of Fallot </li></ul><ul><li>Transposition of the Great Arteries </li></ul><ul><li>Tricuspid Atresia </li></ul><ul><li>Ebstein’s Anomaly </li></ul>
  38. 38. Tetralogy of Fallot <ul><li>Pulmonary stenosis </li></ul><ul><li>VSD of the membranous portion </li></ul><ul><li>Overriding aorta </li></ul><ul><li>Right ventricular hypertrophy due to shunting of blood </li></ul>
  39. 39. Tetralogy of Fallot <ul><li>Addition of an atrial septal defect falls in the category of Pentalogy of Fallot. </li></ul><ul><li>Hypoxic spells and squatting. </li></ul><ul><li>Cyanosis and clubbing. </li></ul>
  40. 40. Tetralogy of Fallot
  41. 41. Transposition of the Great Arteries <ul><li>Aorta from right ventricle, pulmonary artery from left ventricle. </li></ul><ul><li>Cyanosis from birth, hypoxic spells sometimes present. </li></ul><ul><li>Heart failure often present. </li></ul><ul><li>Cardiac enlargement and diminished pulmonary artery segment on x-ray. </li></ul>
  42. 42. Transposition of the Great Arteries <ul><li>Anatomic communication must exist between pulmonary and systemic circulation, VSD, ASD, or PDA. </li></ul>
  43. 43. Transposition of the Great Arteries
  44. 44. Transposition of the Great Arteries
  45. 45. Tricuspid Atresia <ul><li>Tricuspid valve is completely absent in about 2% of newborns with congenital heart disease. </li></ul><ul><li>Blood flows from right atrium to left atrium through foramen ovale. </li></ul><ul><li>Early cyanosis. </li></ul>
  46. 46. Tricuspid Atresia <ul><li>Repair consists of shunt from right atrium to pulmonary artery or rudimentary right ventricle (Fontan procedure). </li></ul>
  47. 47. Ebstein’s Anomaly <ul><li>Septal and posterior leaflets of the tricuspid valve are small and deformed, usually displaced toward the right ventricular apex. </li></ul><ul><li>Most patients have an associated ASD or patent foramen. </li></ul><ul><li>Cyanosis and arrhythmias in infancy are common. </li></ul>
  48. 48. Ebstein’s Anomaly <ul><li>Right heart failure in half of patients. </li></ul><ul><li>Operative repair with tricuspid valve replacement. </li></ul>
  49. 49. Congenital Heart Disease <ul><li>The end, thank you. </li></ul>