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Congenital Heart Lesions- Right to Left Shunts

Congenital Heart Lesions- Right to Left Shunts. Zeev Perles MD Pediatric Cardiology Hadassah Jerusalem, 11/2013. Congenital Heart Lesions- Right to Left Shunts. R-L shunting = blue blood contaminating systemic cycle R-L shunting  Cyanotic CHD ???.

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Congenital Heart Lesions- Right to Left Shunts

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  1. Congenital Heart Lesions-Right to Left Shunts Zeev Perles MD Pediatric Cardiology Hadassah Jerusalem, 11/2013

  2. Congenital Heart Lesions-Right to Left Shunts R-L shunting = blue blood contaminating systemic cycle R-L shunting Cyanotic CHD ???

  3. Congenital Heart Lesions-Right to Left Shunts R-L shunting = blue blood contaminating systemic cycle R-L shunting Cyanotic CHD ???

  4. Congenital Heart Lesions-Right to Left Shunts R-L shunting = blue blood contaminating systemic cycle R-L shunting Cyanotic CHD ???

  5. Cyanosis • Kuanosis (Greek) “blueness” • Deoxygenated capillary blood • Lundsgaard & Van Slyke (1923) • ≥3-5 gram% of reduced Hgb • Central or Peripheral • Look at skin, mucosa, nailbeds

  6. Cyanotic CHD Tetralogy of Fallot (TOF) Transposition of the Great Arteries (TGA) Tricuspid valve atresia + Pulmonary aTresia (with VSD or IVS) Total anomalous pulmonary venous return (TAPVR) Truncus arteriosus EbsTein’s Anomaly of the Tricuspid Valve

  7. 100 60 100 60 100 60 80 70 90 R-L shunting = Cyanotic CHD !!! Qp/QS ?

  8. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  9. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  10. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  11. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  12. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  13. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  14. Qp/Qs << 1 Severe desaturation/cyanosis (RA SiO2 60-75%) Oligemic CXR Normal respiratory status Normal growth Need: BT Shunt RV-PA conduit + VSD Qp/Qs >> 1 Mild/No desaturation (RA SiO2 85-99%) CXR – increased PVM’s Tachypneic and dyspneic FTT !!! Need: RV-PA conduit + VSD

  15. Cyanotic CHD • Take-home message: • Some CYANOTIC CHDs are not necessarily blue • The degree of “blueness” depends on Qp/Qs ratio

  16. ונעבור לנציגנו במיון ילדים...

  17. Can You Guess – O2S ? O2S = O2S =

  18. Can You Guess – O2S ? O2S = O2S =

  19. Can You Guess – O2S ? O2S = 80% O2S = 80% !!!

  20. Can You Guess – O2S ? O2S = 80% O2S = 80% !!! Total Hb- 10g%  Deox Hb=10*20%=2 g% Total Hb- 20g%  Deox Hb=20*20%=4 g%

  21. Can You Guess –Hgb g% ?

  22. Can You Guess –Hgb g% ? All- 75% !!!

  23. Can You Guess –Hgb g% ? All- 75% !!! Hgb ???

  24. Can You Guess –Hgb g% ? 18g% !!! 9g% !!! 21g% !!! All- 75% !!! Hgb ???

  25. Can You Guess – O2S ? • Take-home message: • A child with CYANOTIC CHD is not necessarily blue • The degree of “blueness” depends on hemoglobin level

  26. Breathing Patterns

  27. Breathing Patterns Tricuspid Atresia(Qp/QS 1.3) Large VSD+PDA

  28. Can You Guess – O2S ? Tricuspid Atresia Truncus Arteriosus

  29. Medical History Large VSD Tetralogy of Fallot

  30. Can You Guess – O2S ?

  31. Can You Guess – O2S ?

  32. Can You Guess – O2S ?

  33. Can You Guess – O2S ?

  34. Cyanotic CHD- Pulmonary flow obstruction with R-L Shunting Tetralogy of Fallot (TOF) Tricuspid valve atresia (with PS) Pulmonary aTresia (with VSD or IVS) Classic Cyanotic Heart lesions

  35. Cyanotic CHD- Pulmonary flow obstruction with R-L Shunting After birth- closure of arterial duct: Baby dies!!!!

  36. Cyanotic CHD- Pulmonary flow obstruction with R-L Shunting After birth- closure of arterial duct: Baby dies!!!! iv PGE

  37. Blue Baby Syndrome • Helen B. Taussig 1898-1986 • Orphan- age 11 • XX • Severe Dyslexia • Deafness • Founder of Pediatric Cardiology

  38. TOF Natural history

  39. ACQUIRED CONDITIONS • Brain abscesses • most commonly with TOF • rarely before 2 years of age • headaches, fever, seizures, or neurologic defecits • Cerebrovascular accidents • Infectious endocarditis • May be pre-disposed to otitis media

  40. Blue Baby Syndrome

  41. EC S1 Systolic murmur of PS S2 single Physical Exam in Tetralogy of Fallot • Cyanosis - degree depends on PS • Clubbing (>6m) • Normal pulses • Increased RV impulse at RSB • SS2 • Murmur along LSB

  42. CXR • Heart size enlarged • Small RVOT segment+ large RV- Coeur en sabot or boot shaped heart • May see right sided aortic arch • Pulmonary vasculature decreased (oligemic)

  43. Anatomy/Physiology

  44. Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965

  45. Anatomy/Physiology

  46. Anatomy/Physiology

  47. Anatomy/Physiology

  48. Anatomy/Physiology

  49. TOF management • Squatting • Soothing • Sedation • Seline • Slowing the rate • Surgery

  50. TOF management • Squatting • Soothing • Sedation • Seline • Slowing the rate • Surgery

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