1. Vascular Diseases of Lungs

2. Pulmonary Hypertension It is the increase in blood pressure in pulmonary arteries, veins and capillaries. It leads to shortness of breath, dizziness and fainting M:F ratio 1:3 Prevalence 15/million Primary vs secondary

3. Presentation Gradual onset of dyspnea and fatigue Non productive cough Syncope Peripheral edema (ankle swelling)

4. Types Arterial Venous Capillary

5. Pathogenesis Arterial: vasoconstriction due to vascular hyper- reactivity due to endothelial dysfunction (low PG, NO, high endothelin)– thickening and fibrosis of blood vessels – increase pulmonary arterial pressure – increase work load to the right side of the heart – right ventricular hypertrophy – RV failure – less blood to the left side of heart – hypoxemia – systemic congestion of blood (liver, lower ankle swelling, increased jugular veinous pressure)

6. Pathogenesis Venous: no obstruction of blood flow – Due to left side heart failure – pooling and congestion of blood in lungs – increase hydrostatic pressure in pulmonary veins – chronic process – pulmonary hypertension

7. Pathogenesis Capillary: due to lung disease eg. idiopathic pulmonary fibrosis. There is generalized fibrosis of the alveolar walls – changes of the capillary network due to loss and fibrosis – arterialization of capillaries – increase resistance and pressue Generalized hypoxia - vasoconstriction

10. Causes WHO group I: pulmonary arterial hypertension: disease of arteries, idiopathic pulmonary arterial hypertension, vasculitis, autoimmune disease WHO group II: Pulmonary hypertension associated with heart disease WHO group III: Pulmonary hypertension associated with lung disease: COPD, interstitial pneumonia.. WHO group IV: Pulmonary hypertension due to multiple pulmonary thromboembolism WHO group V: Miscellaneous: others eg. Sarcoidosis

11. Diagnosis Dyspnea, syncope Elevated jugular venous pressue Congested liver Ankle edema Clubbing of fingers High pulmonary arterial pressure

12. Pulmonary Thromboembolism

13. Common clinical problem Can be fatal Frequently missed Difficult to diagnose 95% thromboembolism of DVT (popliteal veins and larger veins of lower limb) Predisposing factors for thrombosis

14. Risk factors: Prolonged bed rest, following major surgery, severe trauma, congestive heart failure, contraceptive pills, cancer

15. Presentation 60-80% are asymptomatic 5% cause sudden death 10-15% cause lung infarction, presenting as sudden dyspnea, severe chest pain 3% are multiple and cause pulmonary hypertension

16. Consequences of pulmonary thromboembolism: 1. Fatal if it is large (Saddle embolus) 2. Infarction: small emboli, distal occlusion of pulmonary arteries, can be multiple, wedge shape, hemorrhagic “red” infarct 3. Pulmonary hypertension: multiple, chronic 4. Cor pulmonale

22. Diagnosis Needs high index of suspicion Presentation: pleuritic chest pain, severe, with sudden dyspnea, history of DVT or risk for thrombosis Imaging: x ray, CT scan, V/Q scan