5 Congenital Heart Disease(Chd)

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5 Congenital Heart Disease(Chd)

  1. 2. <ul><li>Atrial septal defect ………… .ASD </li></ul><ul><li>Ventricular septal defect …… VSD </li></ul><ul><li>Patent ductus arteriosus …… .PDA </li></ul><ul><li>Tetralogy of Fallot ………… ..TOF </li></ul>
  2. 3. <ul><li>Atrial septal defect </li></ul><ul><li>ASD </li></ul>
  3. 4. <ul><li>Leornardo da Vinci described the patent foramen ovale in the early 1500s </li></ul><ul><li>“ I have found a perforating channel from left auricle to right auricle” </li></ul><ul><li>In 1875, Karl von Rokitansky provided a superb account of pathological anatomy of the atrial septal defect together with its embryological basis </li></ul><ul><li>He even distinguished between primum and secundum defects </li></ul>
  4. 5. <ul><li>Accounts for 20% of CHD </li></ul><ul><li>Two to three times more common in women </li></ul><ul><li>May go unrecognized for decades </li></ul><ul><li>Unoperated survival beyond age 40-50y is no more than 50% with a subsequent increase in mortality at 6% per year after age 50y </li></ul>
  5. 6. IVC, SVC RA overload RV dialated PA dialated Pulmonary congestion Pulmonary veins LA LV volume decreased Aortic volume decreased Systemic volume decreased ASD  
  6. 7. Hemodynamic changes in ASD <ul><li>L R shunt Pulmonary circulation </li></ul><ul><li> Systemic circulation </li></ul><ul><li>2. Cardiac volume RA, RV, LA </li></ul><ul><li>3. Pulmonary Hypertension </li></ul><ul><li>Eisenmenger syndrome </li></ul>ASD
  7. 8. Why the left atrium is no enlarge with ASD ? LV RV LA RA
  8. 9. <ul><li>Ostium secundum defect </li></ul><ul><ul><li>Most common (60-70%) </li></ul></ul><ul><ul><li>Mid portion of IAS (@ FO site) </li></ul></ul><ul><ul><li>Isolated defect associated with MVP </li></ul></ul><ul><li>Ostium Primum defect </li></ul><ul><ul><li>15-20% of ASDs </li></ul></ul><ul><ul><li>Inferolateral portion of IAS </li></ul></ul><ul><ul><li>Frequently associated with cleft in anterior MV leaflet and MR </li></ul></ul>
  9. 10. <ul><li>Sinus Venosus defect </li></ul><ul><ul><li>5-10% of ASDs </li></ul></ul><ul><ul><li>Superior and posterior in relation to fossa ovalis </li></ul></ul><ul><ul><li>Almost always associated with PAPVD into RA or IVC/SVC </li></ul></ul><ul><li>Coronary sinus defect </li></ul><ul><ul><li><5% of cases </li></ul></ul><ul><ul><li>Located inferior and slightly anterior to the fossa ovalis </li></ul></ul><ul><ul><li>Commonly associated with other defects (eg AVSD) </li></ul></ul><ul><ul><li>Also associated with left sided SVC </li></ul></ul>
  10. 11. <ul><li>Grade II-VI systolic ejection murmur at left upper sternal bord, </li></ul><ul><li>follow by a mid-diastolic flow rumble in tricuspid valve region (relative tricuspid valve stenosis) </li></ul><ul><li>P2 widely split and fixed </li></ul>
  11. 12. <ul><li>ECG : RA dilated, RV hypertrophy, CRBBB,IRBBB </li></ul><ul><li>CXR : cardiomegaly (RA, RV), increased pulmonary </li></ul><ul><li>markings, prominent MPA segment </li></ul><ul><li>Echo : dilated RA, RV plus paradoxical septal motion </li></ul>
  12. 14. <ul><li>Right atrium /ventricle/outflow tract/ pulmonary artery enlarge </li></ul><ul><li>Right ventricle overloading </li></ul><ul><li>Doppler :shunt between atrium </li></ul>
  13. 15. Subcostal four chamber view
  14. 16. <ul><li>Bronchopneumonia </li></ul><ul><li>Arrhythmia </li></ul><ul><li>Eisenmenger syndrome </li></ul>
  15. 17. <ul><li>Anomalous pulmonary drainage </li></ul><ul><li>Pulmonary stenosis </li></ul><ul><li>MV cleft </li></ul>
  16. 18. <ul><li>Anti congestive medications for CHF </li></ul><ul><li>Interventional therapy : transvenous closure with an occluding device is now available in some medical centers </li></ul><ul><li>Surgical options : Large L-R shunt; CHF; Pulmonary congestion </li></ul>
  17. 19. 返回
  18. 24. Amplatzer 双盘样闭合器
  19. 25. After plantation 四腔位 正位
  20. 27. <ul><li>Ventricular septal defect </li></ul><ul><li>VSD </li></ul>
  21. 28. <ul><li>Henri Roger was the first man to describe a ventricular septal defect, in 1879 he wrote: </li></ul><ul><li>“ A developmental defect of the heart occurs from which cyanosis does not ensue in spite of the fact that a communication exists between the cavities of the two ventricles and in spite of the fact that the admixture of venous blood and arterial blood occurs. This congenital defect, which is even compatible with long life, is a simple one. It comprises a defect in the interventricular septum” </li></ul>
  22. 29. <ul><li>Most common CHD in children (25%) </li></ul><ul><li>Isolated VSD found in only 10% of adults with CHD </li></ul><ul><li>75-80% of small VSD’s close spontaneously by late childhood </li></ul><ul><li>10-15% of large VSD’s close spontaneously </li></ul><ul><li>25-40% of defects close before age 3-4, and 90% before age 8 </li></ul><ul><li>Risk factors for decreased survival for unoperated patients include: Cardiomegaly on CXR, Elevated PASP (>50 mmHg), and CV symptoms </li></ul>
  23. 30. VSD classification 1: membranes 2:outflow 3:inflow 4:muscular ① ② ③ ④
  24. 32. IVC, SVC RA RV dialated PA dialated Pulmonary congestion LA overload LV overload Aortic volume decreased Systemic volume decreased VSD   Hemodynamic changes in VSD Pulmonary blood overload
  25. 33. Hemodynamic changes in VSD
  26. 34. Hemodynamic changes in VSD <ul><li>L R shunt Pulmonary circulation </li></ul><ul><li> Systemic circulation </li></ul><ul><li>2. Cardiac volume LA, LV, RV </li></ul><ul><li>3. Pulmonary Hypertension </li></ul><ul><li>Eisenmenger syndrome </li></ul>VSD
  27. 35. Why the left atrium can enlarge with VSD? LV RV LA RA
  28. 36. <ul><li>Depends on size, PVR, associated lesions </li></ul><ul><li>Small-moderate VSD </li></ul><ul><li>Grade II-VI harsh pansystolic murmur </li></ul><ul><li>At lower left sternal border </li></ul><ul><li>Normal P 2 (pulmonic second sound) </li></ul><ul><li>Large shunt VSD without PH (pulmonary Hypertension) </li></ul><ul><li>grade III-VI PSM ,P 2 is normal, </li></ul><ul><li>Congestive heart failure may present </li></ul><ul><li>In present of PH </li></ul><ul><li>P 2 is loud, shot systolic ejection murmur left sternal border </li></ul><ul><li>may be cyanotic if reversal of shunt </li></ul>
  29. 37. In moderate- large VSD CXR: cardiac enlargement and increased PA flow ECG: may variable In large defect CXR:marked cardiomegaly,and increased pulmonary Vascularity ECG: RVH, LVH, or both Echocardiography: defining position and size Doppler echo: estimate the pulmonary systolic pressure
  30. 40. <ul><li>Bronchopneumonia </li></ul><ul><li>Congestive heart failure </li></ul><ul><li>Pulmonary edema </li></ul><ul><li>Subacute bacterial endocarditis </li></ul><ul><li>Eisenmenger syndrome </li></ul>
  31. 41. <ul><li>Small defect may rarely need medical management other </li></ul><ul><li>than prophylaxis for SBE </li></ul><ul><li>Significant spontaneous closure : 25% </li></ul><ul><li>Anti congestive medications for CHF </li></ul><ul><li>Interventional therapy : transvenous closure with an occluding device is now available in some medical centers </li></ul><ul><li>Surgical management: refractory CHF, failure to thrive, </li></ul><ul><li>repeated pneumonia , reversible PH </li></ul>
  32. 43. patch Direct to sow up 返回
  33. 44. Orbit way : FV ->IVC ->RA->RV ->VSD -> LV ->AAO ->DAO ->FA
  34. 47. <ul><li>Patent Ductus Arteriosus </li></ul><ul><li>PDA </li></ul>
  35. 48. <ul><li>15% of CHD </li></ul><ul><li>risks and history that of the shunt magnitude </li></ul><ul><li>small, restrictive--asymptomatic, risk of endocarditis </li></ul><ul><li>moderate to large--heart failure, arterial “steal” </li></ul>
  36. 50. Hemodynamic changes in PDA RA RV PA congestion PA congestion LV dialated Ao Systemic volume decreased PA dialated Descending ao dialatd Ascending ao dialatd PH Diastolic BP decreased PDA LA dialated
  37. 51. Hemodynamic changes in PDA <ul><li>L R shunt Pulmonary circulation </li></ul><ul><li> Systemic circulation </li></ul><ul><li>2. Cardiac volume LA, LV RV </li></ul><ul><li>3. Pulmonary Hypertension </li></ul><ul><li>differential cyanosis </li></ul>PDA
  38. 52. Patent Ductus Arteriosus RA LA RV LV PA Ao
  39. 53. <ul><li>A symptomatic when the ductus is mall, when defect is large, CHF may develop </li></ul><ul><li>A grade I-VI continuous (machinery) murmur audible at ULSB or left infraclavical </li></ul><ul><li>An apical diastolic rumble is audible (large shunt) </li></ul><ul><li>Bounding peripheral pulses with wide pulse pressure </li></ul>
  40. 55. <ul><li>EKG findings are similar to those of VSD </li></ul><ul><li>CXR: normal in small PDA, Cardiomegaly in large PDA </li></ul><ul><li>Echocardiography can directly determine the hemodynamic significance </li></ul>
  41. 56. Left atrium and ventricular hypertrophy
  42. 57. 肺多血,肺动脉段
  43. 59. <ul><li>Bronchopneumonia </li></ul><ul><li>Congestive heart failure </li></ul><ul><li>Pulmonary edema </li></ul><ul><li>Subacute bacterial endocarditis </li></ul><ul><li>Eisenmenger syndrome </li></ul>Large shunt PDA: similar to VSD
  44. 60. <ul><li>Indomethacin is effective in infant within one week of age </li></ul><ul><li>Prophylaxis should be used when SBE arised </li></ul><ul><li>Interventional therapy : Catheter closure of PDA with various devices </li></ul><ul><li>Surgical ligation and division without cardiopulmonary bypass is indicated for all PDAs, regardless of size </li></ul>
  45. 63. 蘑菇伞堵塞术(图示) PDA
  46. 67. <ul><li>Tetralogy of Fallot </li></ul><ul><li>TOF </li></ul>
  47. 68. <ul><li>R-to-L shunts: CYANOSIS in 1year after birth (or diagnosis if Tetralogy of Fallot), prevalence 10% of CHD </li></ul><ul><li>Basic abnormality is malalignment of the outflow (infundibular) portion of the ventricular septum </li></ul><ul><li>Pathoanatomy: </li></ul><ul><ul><li>VSD </li></ul></ul><ul><ul><li>aortic over ride </li></ul></ul><ul><ul><li>pulmonary outflow tract stenosis </li></ul></ul><ul><ul><li>right ventricular hypertrophy </li></ul></ul>
  48. 69. <ul><li>Infundibular deviated to anterior, </li></ul><ul><li>superior and left </li></ul><ul><li>1. 50 % RVOTO </li></ul><ul><li>2. 20% - 50 % PS </li></ul><ul><li>3. Superior pulmonary valve stenosis or peripheral pulmonary stenosis or absent left pulmonary artery </li></ul><ul><li>4. Asssociated heart malformation </li></ul><ul><li>40 % inner heart malformation </li></ul><ul><li>20 %- 30 % out heart malformation </li></ul><ul><li>20% right sided aortic arch </li></ul>
  49. 70. Hemodynamic changes in TOF <ul><li>R L shunt </li></ul><ul><li>2. RV volume , LV relatively small </li></ul><ul><li>3. PA flow hypoximia </li></ul>VSD VSD LV AO overriding cyanosis RV PA blood flow decreased
  50. 71. <ul><ul><li>Cyanosis </li></ul></ul><ul><ul><li>Dyspnea </li></ul></ul><ul><ul><li>Squatting position </li></ul></ul><ul><ul><li>Anoxia spells </li></ul></ul>Severe degree of the presentations is related to the degree of right ventricular outflow obstruction.
  51. 72. <ul><li>presentation typically in infancy </li></ul><ul><ul><li>-murmur--present from initial assessment </li></ul></ul><ul><ul><li>-cyanosis if outflow obstruction is severe </li></ul></ul><ul><ul><li>-range of presentation a reflection of the variability of </li></ul></ul><ul><ul><li>outflow obstruction </li></ul></ul><ul><ul><li>-most critical obstructive lesion--pulmonary atresia </li></ul></ul><ul><ul><li>-may be associated with hypoplasia of the pulmonary </li></ul></ul><ul><ul><li>arteries </li></ul></ul>
  52. 73. <ul><li>Hypoxic spell: Occur at 2m - 9m </li></ul><ul><li> -A paroxysm of hyperpnea (rapid and deep </li></ul><ul><li>respiration) </li></ul><ul><li> -increasing cyanosis </li></ul><ul><li> -decreased intensity of the heart murmur </li></ul>
  53. 74. cyanosis
  54. 75. Squatting 蹲踞
  55. 76. Thrombosis
  56. 77. Clubbing
  57. 78. <ul><li>ECG show RA dilated and RV hypertrophy </li></ul><ul><li>CXR: Normal heart size, decreased PVMs, boot-shape heart with a concave MPA segment,right aortic arch is present </li></ul><ul><li>Echocardiography can directly determine the hemodynamic significance </li></ul><ul><li>Cardiac catheterization </li></ul>
  58. 80. Boot -shape
  59. 84. <ul><li>Brain abscess </li></ul><ul><li>Cerebral thromboses </li></ul><ul><li>Subacute bacterial endocarditis </li></ul><ul><li>Congestive heart failure is rare </li></ul>
  60. 85. cyanosis  RVOT contract R  Lshunt  hyperpnea Sat%  Venous return  R  Lshunt  hyperpnea
  61. 86. <ul><li>Keep in a knee-chest position </li></ul><ul><li>Morphine sulfate </li></ul><ul><li>suppress the respiratory Center </li></ul><ul><li>Treat acidosis </li></ul><ul><li>Oxygen inhalation has only limited Calue </li></ul><ul><li>Oral propranolol to prevent the attack </li></ul>
  62. 87. Blalock-Taussig The anastomosis between Subclavian artery and Ipsilateral PA Gor-tex interposition shunt between Subclavian artery and Ipsilateral PA
  63. 88. Conventional repair surgery
  64. 89. <ul><li>Hemodynamic changes </li></ul><ul><li>Clinical features </li></ul><ul><li>Laboratory findings </li></ul><ul><li>Treatment </li></ul>

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