Fibrosis
Intimal fibrosis causes the pulmonary arteries to become stiff and to thicken. As the vessels narrow, they can become completely blocked or obliterated, leading to increased vascular resistance.1,2 Evidence of excess endothelin (ET) has been observed in pulmonary arteries with intimal fibrosis.3
   
Pulmonary Arterial Hypertension
   
 
Clinical Definition4
•  
Mean pulmonary arterial pressure >25 mm Hg
•  
Pulmonary capillary wedge pressure
≤15 mm Hg
•  
Pulmonary vascular resistance >3 Wood units
REFERENCES
1. 
Morrell, N. Pulmonary vascular disease. In: Dilworth JP, Baldwin DR. Respiratory Medicine. Amsterdam: Overseas Publishers Association, Harwood Academic; 2001:199–234.
2. 
McCance KL, Huether SE. Pathophysiology: The Biologic Basis for Disease in Adults and Children. 4th ed. St. Louis, MO: Mosby; 2002:1133.
3. 
Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med. 1993;328:1732–1739.
4. 
McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009;53:1573–1619.