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PPHN PPHN is defined as the failure of normal circulatory - PowerPoint PPT Presentation

Feb 13, 2023 •302 likes •1.64k views

Dr .P.K. Rajiv MBBS DCH MD Fellowship in Neonatology ( Australia) Head of Newborn Services NMC Specialty Hospital Dubai United Arab Emirates Formerly Professor and Head of Neonatology Amrita Institute Of medical Sciences Cochin Kerala PPHN PPHN

  1. Dr .P.K. Rajiv MBBS DCH MD Fellowship in Neonatology ( Australia) Head of Newborn Services NMC Specialty Hospital Dubai United Arab Emirates Formerly Professor and Head of Neonatology Amrita Institute Of medical Sciences Cochin Kerala

  2. PPHN PPHN is defined as the failure of normal circulatory transition,that occurs after birth.It is a syndrome characterised by marked pulmonary hypertension that causes hypoxemia and right to left shunting of blood.The clinical clue is the labile hypoxemia out of proportion to the disease process.

  3. PPHN

  4. DIAGNOSIS OF PPHN PATHOGENESIS MANAGEMENT POST INO ERA

  5. Persistent Pulmonary Hypertension

  7. Pulmonary Hypertension Outline

  8. Neonatal Respiratory Failure

  9. PPHN: A Clinical Syndrome

  10. Etiology of HRF

  11. Not Enough Oxygen In • Apnea – neurologic and pharmacologic causes • Diffusion barrier – RDS, aspiration, pneumonia • Obstruction – pneumothorax, head position

  12. Oxygen “mal-absorption” • Shunting lesions – cardiac – non-cardiac (like PPHN) • Hematologic – methemoglobinemia – carboxyhemoglobinemia

  13. Too Much Oxygen Out • High oxygen consumption – Sepsis – Low flow, high extraction • acrocyanosis • hyperviscosity/polycythemia • extravasated (ie bruising)

  14. Vascular Pathogenesis of HRF

  15. Hemodynamic Changes in HRF

  16. Common Associations with PPHN

  17. Pulmonary Vascular Resistance is Increased in Fetal Life

  18. Variations in PVR and SVR During Gestation Human Fetus

  19. Normal Fetus

  20. Mechanisms of Increased Pulmonary Vascular Resistance in Fetal Life

  21. Dilation of Pulmonary Blood Vessels at Birth

  22. Pulmonary Vascular Resistance Falls at the Time of Birth

  25. Regulation of Pulmonary Vascular Tone

  26. PPHN new modalities of treatment

  27. PPHN new modalities of treatment

  28. PPHN new modalities of treatment

  29. Nitric oxide

  30. Maturation of the NO-c GMP System

  31. Nitric Oxide is a Byproduct of the Conversion of Arginine to Citrulline

  32. Pathogenesis of PPHN

  33. eNOS: A Double Edged Sword

  34. eNOS, Heat Shock Protein 90 & Superoxide radical(O2-)

  35. Pathology of PPHN

  36. PPHN & Distribution of Muscle

  37. Pathophysiology of Pulmonary Hypertension

  38. Diagnosis of PPHN

  39. Cyanosis

  40. Hyperoxia Test • Infant on Room Air, get ABG • Infant on 100% oxygen, get ABG • PaO 2 unchanged = fixed shunt = CCHD • Max PaO 2 <100 = CCHD • Max PaO 2 >200 = No CCHD

  41. Hyperoxia Test • Jones: 1976 – 8/109 with CCHD had PaO 2 > 100mmHg – 7/23 without CCHD (bad RDS etc) had PaO 2 < 150mmHg • Hypoplastic Left Heart Syndrome > 300mmHg • TGA, TAPVR > 200mmHg • Don’t be fooled by early high PaO 2 s

  42. Hyperoxia Test • Don’t do the room air part – Looking for minimal PaO 2 change from 21% to 100% fiO 2 – Hyperoxia test developed pre pulse-ox – With pulse-ox you can tell when PaO 2 s are not changing despite big changes in fiO 2 (for sats that are between 70 and 95%) – Probably the norm to have some degree of lung disease at the time of the test anyway

  43. Shunt Curves • Hyperoxia Proper • Hyperoxia CPAP • Hyperoxia hyper- ventilation

  44. Thumb Rule to Assess Shunt / PPHN • Fio 2(%) x 4 optimum pao2 • Fio2(%) x 3 acceptable pao2 with shunt • Any value of pao2 exceeding 15 to 20 % of this value is a significant shunt

  45. Information Needed • Clinical appearance – “comfortably tachypneic and blue” • Pulses/perfusion – differential, delayed • Pulse-Ox/ABG – pre and post ductal, max PaO 2 • Auscultation – S2, Murmur

  46. Information Needed • CXR – heart shapes • snowman = TAPVR 1 • boot = pulm atresia, TOF, tricuspid atresia • egg on string = TGA + /- pulmonary vascularity • EKG – axis – increased or decreased forces • ECHO – the most important test in PPHN

  47. Echocardiographic Diagnosis of PPHN

  48. PDA with Right to Left Shunt

  49. Novel Methods for Assessment of Right heart Structure and Function in Pulmonary Hypertension

  50. Novel Methods for Assessment of Right heart Structure and Function in Pulmonary Hypertension

  51. Novel Methods for Assessment of Right heart Structure and Function in Pulmonary Hypertension

  52. Accuracy of clinical diagnosis and decision to commence intravenous prostaglandin E1 in neonates presenting with hypoxemia in a transport setting

  53. Use of Intravenous PGE 1 in Neonates Presenting with Hypoxemia

  54. Management of Infants with Pulmonary Hypertension

  55. Control of Blood Pressure DOPAMINE 10 DOBUTAMINE 10 MILRINONE Feel posterior tibial pulsation well

  56. Control of FRC CPAP / PEEP XRAY aim for about 8.5 to 9 ribs expansion clearance of haziness

  57. DONT BASH THE LUNG Ph . > 7.25 Co2 < 60 mmhg O2 50 - 70 mmhg Pediatrics oct 1985 76 (4 ) 488 -94 Wung JT DO SO ONLY IF THE END EXPIRATORY PRESSURE OR CPAP IS RIGHT

  58. Cardiopulmonary Interactions in PPHN

  59. The Vicious Cycle of PPHN

  60. Unproven Therapeutic Strategies in PPHN

  61. Proven Therapeutic Strategies in PPHN

  62. Use of Surfactant in PPHN

  63. Surfactant and Meconium Aspiration Syndrome: Mechanisms of Action

  64. Use of Surfactant in PPHN Oxygenation Index

  65. Surfactant Replacement in the Term Newborn

  66. Guidelines for Mechanical Ventilation in PPHN

  67. Effect of Ventilation – Pulmonary Vascular Resistance (PVR) is Minimal at FRC

  68. The million dollar question of optimisation of PEEP

  69. Randomized Multicenter Trial of Inhaled NO and High Frequency Oscillatory Ventilation in Severe PPHN

  70. Randomized Multicenter Trial of Inhaled No and High Frequency Oscillatory Ventilation in Severe PPHN

  71. Guidelines for Arterial Blood Gases in PPHN

  72. Pulmonary Vascular Resistance & pH

  73. Neonatal Lambs

  74. Model – PPHN with Remodeled Pulmonary Vasculature

  75. Severe Hypoxic Pulmonary Vasoconstriction in Lambs with PPHN; Change Point – Similar to Control Lambs

  76. Oxygen Saturation and PVR

  77. Use of Supplemental Oxygen in PPHN PO 2 mmHg

  78. Pulmonary Vascular Resistance % Increase PVR

  79. Changes in Pulmonary Vascular Resistance in Lambs Ventilated with 21% or 100% O 2

  80. Changes in Pulmonary Vascular Resistance in Lambs PPHN Ventilated with 21%, 50% or 100% O 2

  81. Changes in Pulmonary Vascular Resistance in Lambs PPHN Ventilated with 21%, 50% or 100% O 2

  82. Nitric Oxide and Superoxide Radical

  83. Combinational effects of SOD and NO in lambs with PPHN

  84. Regulation of Pulmonary Vascular Tone

  85. Nitric Oxide

  87. Guidelines for Using NO

  88. Initiation of INO and ECMO

  89. Response Rate by Diagnoses

  90. Mechanisms for Poor NO Response

  91. Inhaled NO vs Control: Outcome Requirement for ECMO

  92. Comparison Inhaled NO vs Control, Outcome Death

  93. Inhaled NO vs Control: Outcome Neurodevelopmental Disability at 18 to 24 Months Aamong survivors

  94. Discontinuing Nitric Oxide

  95. No Levels Before Stopping Treatment

  96. Oxygenation Index Oxygenation Index

  97. Post Nitric Oxide Era

  98. Post – INO Era

  99. Use of Sildenafil in PPHN

  100. Viagra used first time in the world successfully in severe PPHN Dr Rajiv and team June 2002

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