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Increased vascular resistance associated with endothelin (ET)-mediated arteriopathy in PAH forces the right ventricle of the heart to work harder. Over time, this leads to right ventricular hypertrophy, reduced contractility, and reduced blood flow to the right ventricular myocardium.1 Acute or chronic ischemia may result.1 The strain and dysfunction experienced by the right ventricle in the late stages of PAH commonly lead to right heart failure.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; 2001:1919. |
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2. |
McCance KL, Huether SE. Pathophysiology: The Biologic Basis for Disease in Adults and Children. 4th ed. St. Louis: Mosby; 2002:1133. |
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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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© 2008 Actelion Pharmaceuticals US, Inc. All rights reserved. |
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