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Pulmonary hypertension and operability
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Pulmonary hypertension and operability

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A talk given at the Dept of Pediatrics Lusaka University teaching Hospital. Basic and clinical approach to early operability to prevent pulmonary hypertension. Included is clinical management of ...

A talk given at the Dept of Pediatrics Lusaka University teaching Hospital. Basic and clinical approach to early operability to prevent pulmonary hypertension. Included is clinical management of Pulmonary Hypertension.

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Pulmonary hypertension and operability Presentation Transcript

  • 1. PulmonaryHypertension
  • 2. Best Method forMx of PHTN isPREVENTION
  • 3. Conditions associated withPAH Acyanotic CHD  Increased Pulmonary Blood Flow Cyanotic CHD  Increased Pulm Blood Flow
  • 4. CLASSIFICATION OF CHD ACYANOTIC  CYANOTIC Increased PBF  Decreased Flow  ATRIAL: ASD  TOF  VENTR: VSD  Pulm Atresia  ARTERIAL: PDA  Increased Flow  COMBINED:  TAPVD VSD+PDA  TGA No Shunts  Truncus  Pulm or Aortic  Tricuspid Atresia Stenosis
  • 5. So what is theright time tooperate in theseconditions
  • 6. Timing of surgery: Acyanotic ASD: 2 years or later VSD  Large: 3-6 months  Moderate: when there is FTT  Small: when there is AI or InfectiveEndocardiaits
  • 7. Acyanotic, when to operate PDA  Infancy  ALL PDA’S CAN BE CLOSED WITH DEVICE  Neonatal  Prematurity  Closure by surgical ligation  Full Term  Wait for child to grow if possible
  • 8. ATRIAL SEPTAL DEFECTPRIMUM SINUS VENOSUS
  • 9. ATRIAL SEPTAL DEFECT-II
  • 10. ATRIAL SEPTAL DEFECT-II
  • 11. ASD-DEVICE
  • 12. Acyanotic CHD Increased Pulmonary Blood Flow  PRETRICUSPID SHUNT: RA RV DILATATION  ATRIAL SEPTAL DEFECT  POST TRICUSPID SHUNT: LA V DILATATION  VENTRICULAR SEPTAL DEFECT PATENT DUCTUS ARTERIOSUS
  • 13. What is aLarge, Moderate, Small VSD Effects of VSD  PRESSURE EFFECT: Pulmonary Hypertension  VOLUME EFFECT: Cardiac enlargement
  • 14. Pressure Effect: Types Flow Related PAH: Reversible Irreversible PAH due to permanent Changes
  • 15. Flow Related PAH Increased Flow Increased Pressure When you remove the extra flow ie close the VSD, the Pulmonary Pressure comes back to normal
  • 16. Flow related PAH (Pre/PostTricuspid Shunt) Symptoms of increased Flow  Tachypnea, Rec infections, failure to thrive Signs  Tachycardia, Harrison’s sulcus, retractions X-ray  Cardiac enlargement, Increased Pulmonary Blood Flow
  • 17. Increased Flow PAH All these indicatePatient is operable with good results without post-op PAH
  • 18. Till when is this phase: Reversible PAH VSD-LARGE: UPTO 6 MONTHS PDA-LARGE: UPTO 6 MONTHS ASD: LARGE: UPTO LATER 4-8 YRS
  • 19. So, What is a Large Shunt Post tricuspid Large VSD/PDA  Clinically PAH Present (Pressure Effect)  Clinically Volume Effect Present (Cardiac Enlargement)
  • 20. Moderate Shunt No Pressure Effect But Volume Effect Present
  • 21. Small Shunt No Pressure or Volume Effect No Symptoms or Signs of increased flow
  • 22.  So if the surgery is done at the right time it is likely the patient will not get pulmonary hypertension
  • 23. What Happens when Reversible PAH starts becoming Irreversible …the child shows some signs and these are signs of Post Op PAH
  • 24. Signs of Reversible toIrreversible PAH Symptoms:  Start improving  Less FTT  Less Infectios  Less tachypnea Signs:  Murmur shorter, P2 Louder, Cardiac Enlargement less
  • 25. When Reversible Changing toIrreversible Patient still operable Butthe post op risks are more and episode of life threatening PAH in immediate post op period is high
  • 26. When Completely Irreversible Patient now has Eisenmanger’s Decreased Pulm Blood Flow Cyanosis starts Now risk of surgery more than living without surgery
  • 27. Chest XraysIndicatingIncreased PBFw PAH ieoperability
  • 28. VSD
  • 29. ASD
  • 30. MODERATE VSD
  • 31. LARGE VSD LARGE SHUNT
  • 32. AV CANAL
  • 33. TGA
  • 34. TRUNCUS
  • 35. EISENMANGERS
  • 36. PulmonaryHypertension
  • 37. ClassificationGroup 1 PAH Examples: "Pulmonary arterial hypertension".  1. Idiopathic (IPAH)  2. Familial (FPAH)  3. Associated with (APAH):  Collagen vascular disease  Congenital systemic-to-pulmonary shunts  Portal hypertension  HIV infection  Drugs and toxins  Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy)  4. Associated with significant venous or capillary involvement  Pulmonary veno-occlusive disease (PVOD)  Pulmonary capillary hemangiomatosis (PCH)  5. Persistent pulmonary hypertension of the newborn
  • 38. Classification Group 2 PH — "Pulmonary venous hypertension". Examples:  1. Left-sided atrial or ventricular heart disease  2. Left-sided valvular heart disease Group 3 PH — "Pulmonary hypertension associated with disorders of the respiratory system or hypoxemia". Examples:  1. Chronic obstructive pulmonary disease  2. Interstitial lung disease  3. Sleep-disordered breathing  4. Alveolar hypoventilation disorders  5. Chronic exposure to high altitude  6. Development abnormalities
  • 39. Classification Group 4 PH — "Pulmonary hypertension caused by chronic thrombotic or embolic disease". Examples:  1. Thromboembolic obstruction of proximal pulmonary arteries  2. Thromboembolic obstruction of distal pulmonary arteries  3. Non-thrombotic pulmonary embolism (tumor, parasites, foreign material) Group 5 PH — These patients have PH caused by inflammation, mechanical obstruction, or extrinsic compression of the pulmonary vasculature (eg, sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels by adenopathy, and fibrosing mediastinitis).
  • 40. Histologically Speaking Theabove mechanisms all cause small muscular arteries and arterioles to undergo intimal hyperplasia and medial hypertrophy 1 Narrowed lumen Decreased cross-sectional area Increased resistance 1 - Though again with PPH likely primary process, rather than reactive
  • 41. PULMONARY VASODILATION HYPEROXIA HYPOCARBIA ALKALOSIS NON REM SLEEP SEDATED PARALYSED
  • 42. Basic 3 Mechanisms 2º pulmonary arterial hypertension:  Reduced cross-sectional area of pulmonary vasculature, secondary to:  Occlusion of vessels (e.g. emboli)  Primary disease of pulmonary vasculature walls (e.g. 1º pulmonary hypertension, portal hypertension)  Primary parenchymal disease (e.g. interstitial lung disease, emphysema)  Vasoconstriction 2/2 hypoxia or acidosis  Increased flow through pulmonary vascular bed secondary to left to right shunts  Increased ―back pressure‖ secondary to pulmonary venous hypertension
  • 43. 3 types of abnormalities Maladaptation Maldevelopment Underdevelopment
  • 44. Maladaptation Prototype: Meconium aspiration pneumonia Pneumonia, RDS Obstruction of the airways Chemical pneumonitis Release of endothelin,thromboxane vasoconstrictors
  • 45. Maldevelopment Prototype: Idiopathic PPHN (―black lung‖ PPHN) Vessel wall thickening Smooth muscle hyperplasia Cause – intrauterine exposure to NSAID constriction of ductus arteriosus genetic
  • 46. Maldevelopment Disruption of NO-cGMP pathway Disruption of PGI2-cAMP pathway Guanylate cyclase is less active Increased ROS (reactive oxygen species) vasoconstrictor Increased thromboxane, endothelin
  • 47. Underdevelopment Prototype: Congenital diaphragmatic hernia Pulmonary hypoplasia Decreased cross sectional area of pulmonary vasculature Decreased pulmonary blood flow Abnormal muscular hypertrophy of the pulm arterioles
  • 48. MEDIATORS OF PULMONARYHYPERTENSION Prostacycline Thromboxane A2 Endothelin-1 Nitric Oxide (NO) Serotonin Adrenomedullin Vasoactive Intestinal Peptide (VIP) Vascular Endothelial Growth Factor (VEGF)
  • 49. ENDOTHELIN-1 Potent vasoconstrictor Stimulates proliferation of smooth muscle cells in PA Plasma levels increased in PHT Level inversely proportional to pulmonary blood flow & CO - ? Direct effect
  • 50. VASODILATORS Oxygen CCBs Endothelin-receptor antagonists BNP Calcitonin gene-related peptide
  • 51. Bosentas
  • 52. ENDOTHELIN RECEPTORANTAGONISTS Endothelin-1 overexpressed in PHT Improvepulmonary haemodynamics, exercise capacity, functional status, clinical outcomes Bosentas, sitaxentan and ambrisentan
  • 53. BOSENTAS Sulphonamide-based ETA & ETB receptor blocker Inducer of  CYP2C9 - Vori/ fluconazole, warfarin, digoxin, simvastatin, t ac/ sirolimus, sildenafil, OCP  CYP3A4 – ketaconazole  t½ 5.6 +/- 1.6 hours
  • 54. PHOSPHODIESTERASEINHIBITORS Sildenafil  PDE type5 inhibitor  Reduce metabolism of cGMP  t½ 3-5 hours  CYP3A4 & 2C9 substrate  Concentration increased by concurrent bosentan – I/As nitrates Tadalafil  t½ 17 hours  CYP 3A4
  • 55. PROSTACYCLINE ANALOGUES Vasodilators  Reduce R & L afterload & increase SV & CO Platelet aggregation inhibitors Main ADRs  H/A and dizziness (~80%)  Nausea and jaw pain
  • 56. PROSTACYCLINE ANALOGUES Iloprost  IV or Inhaled  I/As with CCBs, BBs and ACEIs (animal data)  NO PK STUDIES FOLLOWING INHALATION!!  t½ ~ 0.7 hours Treprostinol  IV or s/c injection  No CYP inhibition - ? induction  t½ 2-4 hours
  • 57.  Epoprostenol  Continuous IV infusion  F 0.2/ t½ 2-6 mins  Spontaneous B/D to 6-oxo-prostaglandin F1α
  • 58. WHERE TO NOW? PDE5 inhibitors & ERAs first line for 1oPHT Increasingevidence that combination therapies are more effective (theoretical)
  • 59. Nitric Oxide Selective pulmonary vasodilation, improves oxygenation ↑ cGMP Used in ARDS, PPHN, cardiogenic shock, post CPB Risks: methemoglobinemia and carboxyhemoglobinemia, rebound pulm HTN when stopped Requires closed inhalational circuit
  • 60. Phosphodiesterase inhibitors Inhibition of nitric oxide degradation Sildenafil (PDE-5 inhibitor): ↓ PAP/PVR  Min effects on systemic vasculature  Synergistic with NO  Reduction in RV mass: role in prevention or reversal of remodeling of RV Milrinone (PDE-3 inhibitor): ↓ PVR/PAP/SVR in setting of CV shock  Nebulized minimizes systemic vasodilation
  • 61. Prostacyclins Potentpulm and systemic vasodilators with antiplatelet properties  Epoprostenol (IV): ↓ PVR, better CO/ex. Tolerance  s/e: ↓BP, need for central line (risk of infection)  Beraprost (PO): Longer duration  Iloprost (nebulized)
  • 62. Endothelin receptorantagonists Endothelin-1: neurohormone that causes pulm vasoconstriction, smooth muscle proliferation, fibrosis  Stimulates endothelin receptors A & B  A: vasconstriction  B: vasodilation  Nonselective: Bosentan  A selective: sitaxsentan, ambrisentan  Chronic pulm htn tx given long ½ life and no IV preparation  s/e: hepatic toxicity
  • 63. BOSENTAS
  • 64. BOSENTAS
  • 65. BOSENTAS
  • 66. BOSENTAS
  • 67. BOSENTAS
  • 68. BOSENTAS
  • 69. BOSENTAS
  • 70. BOSENTAS
  • 71. BOSENTAS
  • 72. BOSENTAS
  • 73. BOSENTAS
  • 74. DEFINITION Pulmonary hypertension is mean pulmonary artery pressure greater than 25mmHg at rest or greater than 30mmHg with exercise