Because pulmonary hypertension is a complex condition that can result from a wide variety of factors, it shows up in many different kinds of patients. Pulmonary hypertension -- high blood pressure in the arteries supplying blood to the lungs -- has been diagnosed in newborns and in older people, in men and women, and in people with other serious health problems as well as those who have no other diseases.

Pulmonary arterial hypertension (PAH) can strike at any age, although it occurs most often among people 20 to 40 years old, according to the National Institutes of Health. The disease – also known as primary pulmonary hypertension -- affects people of all racial and ethnic origins equally. During childhood, it affects both males and females equally. After puberty, PAH strikes women almost twice as often as men. Why that gender difference exists is a mystery.

The cause of some cases of pulmonary arterial hypertension is unknown, but researchers do know that the condition can run in families. About 6 percent of patients diagnosed with pulmonary arterial hypertension are found to have at least one other family member with the disease. Because of this inherited risk, experts may recommend that all first-order relatives of a patient (siblings, children, and parents) be screened for the disease. The recommended test is an echocardiogram, which is relatively simple and painless. A device that resembles a microphone is placed against the chest. Using sonic waves, the device creates a moving picture of the heart. By studying the image, doctors can estimate pulmonary artery blood pressure. They can also detect changes characteristic of pulmonary hypertension, such as an enlarged right ventricle in the heart.

Appetite suppressants -- most recently, fenfluramine and dexfenfluramine -- have also been definitely associated with the onset of the disease, as has the use of street drugs such as cocaine. Congenital heart disease – heart disease that shows up at birth – can affect the way blood flows through the chambers of the heart, causing elevated pulmonary blood pressure. This is usually diagnosed in childhood, but may go undetected until later in life. Other possible causes of PAH include HIV infection and certain liver or thyroid diseases.

Experts have speculated that people who develop the disease have blood vessels that are abnormally sensitive to certain factors, including cocaine, HIV/AIDS, and a form of human herpes, HHV-8 (one that targets blood vessels and is unrelated to the herpes that causes cold viruses). Researchers at the University of Colorado Health Sciences Center in Denver, for example, reported that some patients with pulmonary hypertension were infected with HHV-8.

Because it is the result of other medical conditions, including heart and lung diseases that often take decades to develop, secondary pulmonary hypertension (SPH) tends to occur later in life than primary pulmonary hypertension. A wide range of underlying conditions can lead to SPH, including chronic obstructive pulmonary disease, sleep disorders such as sleep apnea, sickle cell disease, diseases that cause lung scarring, mitral valve disease, blood clots, collagen vascular diseases such as scleroderma or rheumatoid arthritis, and tumors that press on the pulmonary blood vessels.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References

Last updated August 18, 2009
Copyright © 2003 Consumer Health Interactive

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