7. Pathogenesis
Secondary forms of pulmonary
hypertension.
Endothelial cells play an important role
Increased shear and mechanical injury
associated with
- Left to right shunts → ASD,VSD
- Biochemical injury produced by fibrin
in thromboembolism.
8. In Primary Pulmonary Hypertension;
Endothelial cell injury is idiopathic
May be associated with
- autoimmune disorders (collagen
diseases)
9. - Toxic substances
- Genetic determinants (family history)
- Decreased prostacylin
- Decreased NO
- Increased endothelin
All these promote pulmonary vasoconstriction
11. Endothelial activation
→ endothelial cells thrombogenic
→ promotes the persistence of fibrin
Cytokines
GF → these induce the migration and
replication of vascular smooth muscle cells →
elaboration of extracellular matrix
12. Some patients have vasospastic
component
- Widespread and sustained hypoxic -
vasoconstriction and alveolar
hyperventilation → Pulmonary
hypertension eg high altitude
13. Pathologic obesity (Pickwickian disease)
Upper air way disease eg. Tonsillar
hypertrophy
Neuromascular disease eg. Osteomyelitis
Severe kyphosis
14. Pathologic changes
• May lead to cor-pulmonale
• Pathological changes are seen in Right heart
and pulmonary arterial tree in the lungs
• Right- ventricular hypertrophy
• Right- atrial dilatation
15. Vascular changes
• 1) Arterioles and small pulmonary arteries
Medial hypertrophy
Thickening and redublication of elastic
laminae
Plexiform pulmonary arteriopathy
16. 2) Medium sized pulmonary arteries
concentric intimal thickening
Medial hypertrophy
Adventitial fibrosis
Thickening and redublication of elastic
laminae
3) Large pulmonary arteries
Atheromatous deposits