|
|
 |
 |
 |
|
|
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 (PAH) |
|
|
| |
|
Clinical Definition4,5 |
|
| |
• |
Mean pulmonary arterial pressure >25
mm Hg at rest (>30 mm Hg with exercise) |
|
| |
• |
Normal pulmonary capillary wedge pressure (PCWP) |
|
| |
• |
Associated with arteriopathy and resultant
right ventricular strain |
|
|
|
|
|
|
|
|
REFERENCES |
1. |
Dilworth JP, Baldwin DR. Respiratory Medicine. Amsterdam: Overseas Publishers Association, Harwood Academic; 2001:202–203. |
|
2. |
McCance KL, Huether SE. Pathophysiology: The Biologic Basis for Disease in Adults and Children. 4th ed. St. Louis: 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. |
Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719–725. |
|
5. |
Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336:111–117. |
|
|
© 2008 Actelion Pharmaceuticals US, Inc. All rights reserved. |
|
|
|
|
|
 |
