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Causes
By Mayo Clinic staff
Your heart has two upper and two lower chambers. Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery). In your lungs, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then flows through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart.
Ordinarily, the blood flows easily through the vessels in your lungs, so blood pressure is usually much lower in your lungs. With pulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries. These changes cause extra tissue to form, eventually narrowing or completely blocking the blood vessels, making the arteries stiff and narrow. This makes it harder for blood to flow, raising the blood pressure in the pulmonary arteries.
Idiopathic pulmonary hypertension
When an underlying cause for high blood pressure in the lungs can't be found, the condition is called idiopathic pulmonary hypertension (IPH).
Some people with IPH may have a gene that's a risk factor for developing pulmonary hypertension. But in most people with idiopathic pulmonary hypertension, there is no recognized cause of their pulmonary hypertension.
Secondary pulmonary hypertension
Pulmonary hypertension that's caused by another medical problem is called secondary pulmonary hypertension. This type of pulmonary hypertension is more common than idiopathic pulmonary hypertension. Causes of secondary pulmonary hypertension include:
- Blood clots in the lungs (pulmonary emboli)
- Chronic obstructive pulmonary diseases, such as emphysema
- Connective tissue disorders, such as scleroderma or lupus
- Sleep apnea and other sleep disorders
- Congenital heart disease
- Sickle cell anemia
- Chronic liver disease (cirrhosis)
- AIDS
- Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs' air sacs (interstitium)
- Left-sided heart failure
- Living at altitudes higher than 8,000 feet (2,438 meters)
- Climbing or hiking to altitudes higher than 8,000 feet (2,438 meters) without acclimating first
- Use of certain stimulant drugs, such as cocaine
- McLaughlin VV, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Journal of the American College of Cardiology. 2009;53:1573.
- Pulmonary hypertension. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=11076. Accessed Dec. 16, 2009.
- Pulmonary arterial hypertension. Genetics Home Reference. http://ghr.nlm.nih.gov/condition=pulmonaryarterialhypertension. Accessed Dec. 16, 2009.
- Badesch DB, et al. Diagnosis and assessment of pulmonary hypertension. Journal of American College of Cardiology. 2009;54:S55.
- Austin ED, et al. Genetics and mediators in pulmonary arterial hypertension. Clinical Chest Medicine. 2007;28:43.
- Simonneau G, et al. Updated clinical classification of pulmonary hypertension. Journal of the American College of Cardiology. 2009;54:S43.
- Badesch DB, et al. Medical therapy for pulmonary arterial hypertension: Updated ACCP evidence-based clinical practice guidelines. Chest. 2007;131:1917.
- Keogh AM, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. Journal of the American College of Cardiology. 2009;54:S67.
- Letairis (prescribing information). Foster City, Calif.: Gilead Sciences, Inc.; 2009. http://www.letairis.com/downloads/Full_Prescribing_Information_LET2585.pdf. Accessed Dec. 16, 2009.
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