Pulmonary Hypertension

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Pulmonary Hypertension

  1. 1. Pulmonary Hypertension Dr.CSBR.Prasad, M.D. CSBRP-Dec-2012
  2. 2. Pulmonary HypertensionPulmonary BP is only ⅛ of systemic BPDef: Mean pulmonary arterial pressure exceeding ¼ of systemic levels is PHT CSBRP-Dec-2012
  3. 3. Pulmonary HypertensionClassification:Primary: Idiopathic, no known cause, 6% - have family history (AD)Secondary: Structural abnormalities in cardiopulmonary area CSBRP-Dec-2012
  4. 4. Pulmonary HypertensionClassification:Primary: Idiopathic, no known cause6% - have family history (AD-vp)Mutations in BMPR2 (bone morphogenic protein receptor type-2) CSBRP-Dec-2012
  5. 5. Pulmonary HypertensionPrimary: Idiopathic, Mutations in BMPR2BMPR2 belongs to TGF-ß receptor superfamilyBMP-BMPR2 signalling play a role in: - Apoptosis - Cell proliferation & differentiation - Embryogenesis CSBRP-Dec-2012
  6. 6. Pulmonary HypertensionBMP-BMPR2 signalling in vascular smooth muscle: - Inhibits proliferation - Induces apoptosisHence mutations in this gene causes proliferation of smooth muscle in the vessels CSBRP-Dec-2012
  7. 7. Pulmonary HypertensionHow this gene gets deleted / rendered useless?TWO hit hypothesis:First hit: genetic loss of one locusSecond hit: Environmental factor Mutations in the other gene Environmental factors: disruption of vasoregualtory mechnisms involving Endothelin, Prostacyclin synthase or ACE. CSBRP-Dec-2012
  8. 8. Modifier genes are The nature of thethose that control environmental factorsvascular tone, e.g., remains unknown,endothelin, but presumably, theyprostacyclin cause dysfunction ofsynthetase, and vasoregulatoryangiotensin- mechanismsconverting enzymes CSBRP-Dec-2012
  9. 9. Pulmonary HypertensionClassification:Secondary: Structural abnormalities in cardiopulmonary area• COPD / interstitial lung disease• Congenital / acquired heart disease• Recurrent thromboembolism• Autoimmune diseases CSBRP-Dec-2012
  10. 10. Pulmonary HypertensionSecondary: Structural abnormalities in cardiopulmonary areaEndothelial cell dysfunction is produced by the process that initiates the disorder, such as:The increased shear and mechanical injury associated with left-to-right shuntsThe biochemical injury produced by fibrin in thromboembolismDecreased elaboration of prostacyclin, decreased production of nitric oxide, and increased release of endothelin all promote pulmonary vasoconstriction CSBRP-Dec-2012
  11. 11. Pulmonary HypertensionMorphology:The arterioles and small arteries (40 to 300 µm in diameter) are most prominently affected1. Medial hypertrophy and2. Intimal fibrosis3. Plexogenic pulmonary arteriopathy CSBRP-Dec-2012
  12. 12. CSBRP-Dec-2012
  13. 13. Figure 15-30 Vascular changes in pulmonary hypertension. A, Gross photograph of atheroma formation, a finding usually limited to large vessels. B, Marked medial hypertrophy. C, Plexogenic lesion characteristic of advanced pulmonary hypertension seen in small arteries.CSBRP-Dec-2012
  14. 14. Pulmonary HypertensionClinical course:Primary PHT is more common in femalesBetween 20-40yrsDyspnea and fatigueChest painCyanosisRVHDeath: in 2-5yrs in 80% of the patients due to Cor pulmonale, pneumonia CSBRP-Dec-2012
  15. 15. E N D CSBRP-Dec-2012

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