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In PAH, the pulmonary arteries constrict, resulting in increased resistance to pulmonary blood flow and increased pulmonary blood pressure. Increased expression of endothelin (ET) has been observed in the pulmonary arteries of primary pulmonary hypertension patients, suggesting that excess ET may influence pulmonary vasoconstriction in some cases of PAH.1,2 |
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PULMONARY ARTERIAL HYPERTENSION (PAH) |
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Clinical Definition3,4 |
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Mean pulmonary arterial pressure >25
mm Hg at rest (>30 mm Hg with exercise) |
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Normal pulmonary capillary wedge pressure (PCWP) |
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Associated with arteriopathy and resultant
right ventricular strain |
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REFERENCES |
1. |
Braunwald E, Zipes DP, Libby P, eds. Heart Disease. 2 vols. 6th ed. Philadelphia, PA: WB Saunders Co; 2001:1912. |
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2. |
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. |
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3. |
Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719–725. |
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4. |
Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336:111–117. |
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