Pulmonary Hypertension

Definition

Pulmonary hypertension is a progressive disorder characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs.

Description

Blood is carried from the heart to the lungs by the pulmonary artery. In a healthy individual at rest, the normal blood pressure in a pulmonary artery is approximately 15 mm Hg. In a person with pulmonary hypertension, the pulmonary artery pressure can exceed 25 mm Hg. The condition is very serious and can lead to heart failure and death .

Demographics

The American Heart Association (AHA) estimates that, in the United States, approximately 500–1,000 new cases of pulmonary hypertension are diagnosed every year. The condition affects women twice as much as men.

Causes and symptoms

The increase in pulmonary artery pressure associated with pulmonary hypertension is due to an abnormal narrowing of the small arteries of the lungs.

QUESTIONS TO ASK YOUR DOCTOR

• What is pulmonary hypertension?
• What causes my pulmonary hypertension?
• Can it be cured?
• What does treatment involve?
• Are there surgical options?
• Would oxygen help?

Pulmonary hypertension is classified as idiopathic, meaning that the cause is unknown, or familial, meaning that it is inherited. Together, idiopathic and familial pulmonary hypertension are referred to as primary pulmonary hypertension. The condition is called secondary pulmonary hypertension when triggered by a pre-existing disease, such as:

• Connective tissue diseases, such as scleroderma, sarcoidosis, or systemic lupus.
• Congenital heart or lung disease.
• Liver disease.
• HIV-AIDS.
• Thyroid disorders.
• Abnormal red blood cell disorders (hemoglobinopathies).
• Overproduction of red or white blood cells (myeloproliferative disorders).
• Blood clots in pulmonary arteries (pulmonary embolism).
• Use of legal and illegal drugs, such as appetite suppressants, cocaine, amphetamines.

Symptoms of pulmonary hypertension may include shortness of breath (dyspnea) following exercise or exertion, fatigue, dizziness, fainting , ankle or leg swelling (edema ), bluish lips and/or skin, chest pains.

Diagnosis

The diagnosis of pulmonary hypertension is established on the basis of symptoms combined with a careful patient history to rule out other diseases. Diagnosis relies on tests that measure blood pressure in the pulmonary arteries, and examine heart and lung function. These may include a chest x ray , an electrocardiogram to measure the rate and regularity of the heartbeat, an echocardiogram to obtain images of the heart, and spirometry to measure how well the lungs inhale and exhale air. Genetic testing is also available to screen for the gene mutation that causes idiopathic pulmonary hypertension.

KEY TERMS

AIDS —Acquired immunodeficiency syndrome, caused by HIV infection.

Anticoagulant —A medication that helps prevent blood clots fromforming. Also called a blood thinner.

Calcium channel blockers —Class of blood pressure medications that relax and widen the blood vessels.

Congenital —Present at birth.

Diuretic —A substance that increases the amount of urine passed.

Echocardiogram —Test that uses sound waves to create a moving picture of the heart.

Electrocardiogram (EKG) —Test that can measure the rate and regularity of the heartbeat.

Idiopathic —Disease of unknown cause or origin.

Hemoglobinopathies —Group of disorders affecting the red blood cells that contain hemoglobin, the oxygen carrier molecule.

HIV —The human immunodeficiency virus that causes AIDS.

Hypertension —Abnormally high blood pressure.

Myeloproliferative disorder —A disease in which too many blood cells are made in the bone marrow.

Primary pulmonary hypertension —Pulmonary hypertension that is inherited or occurs for no known reason.

Pulmonary artery —An artery that carries venous blood from the right ventricle of the heart to the lungs.

Pulmonary embolism —A pulmonary embolism occurs when a lung artery is blocked by a blood clot. The blockage is often caused by one or more blood clots that travel to the lungs from another part of the body.

Sarcoidosis —An inflammatory disease characterized by the formation of small nodules of immune cells in the lungs, lymph nodes, and other organs.

Scleroderma —A disease of connective tissue characterized by the formation of scar tissue in the skin and sometimes also in other organs of the body.

Secondary pulmonary hypertension —Pulmonary hypertension that is caused by another medical condition.

Spirometry —Test that measures the air capacity of the lungs.

Systemic lupus —A chronic, inflammatory, connective tissue disease that can affect the joints and many organs.

Treatment

Treatment of pulmonary hypertension is often complex, requiring extensive follow-up care. It seeks to treat the underlying medical disorder that is causing the hypertension. However, it is often the case that curing the pre-existing disease does not cure the pulmonary hypertension. Specific treatment may include medications to help the heart pump blood, diuretics , and medications to lower the hypertension.

Medications specifically approved by the US Food and Drug Administration (FDA) for the treatment of pulmonary hypertension include ambrisentan, bosentan, epoprostenol sodium , iloprost, sildenafil and treprostinil. Calcium channel blockers may also be prescribed in some cases. Many patients are also treated with the anticoagulant warfarin.

Nutrition/Dietetic concerns

A nutritious diet and maintaining a healthy weight can help people with pulmonary hypertension. Diet may include lean chicken, fish, whole grains, and fresh fruits and vegetables. For cardiovascular health, daily calorie intake should be monitored, limiting animal (saturated) and trans fats (hydrogenated oils) to 10% or less.

Therapy

For selected patients who do not respond to medical treatments, a lung transplant may be an option. Some advanced cases may also require oxygen therapy , especially when blood oxygen levels become too low.

Prognosis

Accurate, early diagnosis and prompt treatment have saved the lives of many people with pulmonary hypertension. With optimal therapy, some patients can return to a normal lifestyle.

Prevention

The prevention of pulmonary hypertension includes maintaining a healthy weight and being physically active.

Caregiver concerns

Recent studies suggest that primary pulmonary hypertension should be considered as a diagnosis in elderly patients with unexplained shortness of breath and chest pain . While elderly patients with primary pulmonary hypertension have symptoms common to younger patients with this disease, the initial diagnosis is often incorrect, with the senior patients assumed to have more common diseases of the elderly that cause similar symptoms.

Resources

BOOKS

Hayes, Gale B. Pulmonary Hypertension: A Patient's Survival Guide. Silver Spring, MD: Pulmonary Hypertension Association, 2004.

Hill, Nicholas S., and Harrison W. Farber. Pulmonary Hypertension. New York, NY: Humana Press (Springer Group), 2008.

Parker, James N., and Philip M. Parker, editors. The Official Patient's Sourcebook on Pulmonary Hypertension. San Diego, CA: Icon Health Publications, 2002.

Parker, Philip M. Primary pulmonary hypertension—A Bibliography and Dictionary for Physicians, Patients, and Genome Researchers. San Diego, CA: ICON Group International, Inc., 2007.

PERIODICALS

Braman, S. S., et al. “Primary pulmonary hypertension in the elderly.” Archives of Internal Medicine 151, no. 12 (December 1991): 2433–2438.

Dandapantula, H. K., et al. “Unexplained pulmonary hypertension in elderly patients.” Chest 131, no. 6 (June 2007): 1987–1988.

McArdle, J. R., et al. “Pulmonary hypertension in older adults.” Clinics in Chest Medicine 28, no. 4 (December 2007): 717–733.

OTHER

Pulmonary Arterial Hypertension. NIH Genetics Home Reference, Information Page. (March 20, 2008) http://ghr.nlm.nih.gov/condition=pulmonaryarterialhypertension

Pulmonary Hypertension. American Academy of Family Physicians, Familydoctor Website. (March 20, 2008) http://familydoctor.org/online/famdocen/home/articles/675.printerview.html

What Is Pulmonary Arterial Hypertension? NHLBI, Diseases and Conditions Index. (March 20, 2008) http://www.nhlbi.nih.gov/health/dci/Diseases/pah/pah_what.html

ORGANIZATIONS

American Heart Association (AHA), 7272 Greenville Avenue, Dallas, TX, 75231, (800)AHA-USA-1, http://www.americanheart.org.

American Lung Association (ALA), 61 Broadway, 6th Floor, New York, NY, 10006, (212)315-8700, (800) 548-8252, http://www.lungusa.org.

National Heart Lung and Blood Institute (NHLBI), P.O. Box 30105, Bethesda, MD, 20824-0105, (301)592-8573, (240)629-3246, [email protected], http://www.nhlbi.nih.gov.

Pulmonary Hypertension Association (PHA), 801 Roeder Road, Ste. 400, Silver Spring, MD, 20910, (301)565-3004, (301)565-3994, [email protected], http://www.phassociation.org.

Monique Laberge Ph.D.