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What is Primary Pulmonary Hypertension?

By D. Jeffress
Updated: May 17, 2024

Primary pulmonary hypertension is the medical term for high blood pressure in the arteries that deliver blood to the lungs. A number of genetic and environmental factors are tied to other types of high blood pressure, but the cause of primary pulmonary hypertension is unknown. Warning signs for the condition include shortness of breath, chest pains, and feelings of fatigue. Doctors cannot cure primary pulmonary hypertension, but specialists can provide medications to alleviate symptoms and slow the progression of lung and heart problems.

The lungs receive blood from the right ventricle via the pulmonary arteries and a series of smaller blood vessels. In the lungs, blood is oxygenated and pumped back to the heart so it can be distributed throughout the body. Blood pressure in the right ventricle and pulmonary artery is very low compared to the pressure of oxygenated blood. In the case of primary pulmonary hypertension, however, blood pressure in the lungs increases quickly and remains at an abnormally high level.

Most cases of pulmonary hypertension can be tied to chronic illnesses, blood clots, blood-thinning medications, and a history of heart problems. These conditions can pose serious threats to a person's health, but most of them can be treated to some degree. Primary pulmonary hypertension has no known causes, and is therefore very difficult to treat effectively. Many medical researchers believe that primary cases may be related to genetic conditions, but a direct correlation between genetic mutations and pulmonary hypertension has not been confirmed.

A person who has primary pulmonary hypertension may not experience symptoms in the early stages of the illness. As blood pressure continues to rise, an individual might have difficulties breathing after activity and frequent feelings of fatigue and weakness. The condition eventually results in chronic shortness of breath, heart murmurs, and sharp chest pains. A person who experiences physical symptoms should visit his or her physician as soon as possible to receive a diagnosis.

A doctor can try to pinpoint the causes of chest pain and shortness of breath by taking a chest x-ray and collecting a blood sample to test for autoimmune disorders. If findings suggest pulmonary hypertension, the doctor can refer a patient to a pulmonologist for further testing. The specialist can conduct a computerized tomography scan to carefully examine the lungs and an electrocardiogram to monitor heart activity. He or she may decide to collect a small sample of lung tissue for further analysis. After ruling out all possible causes, the pulmonologist can diagnose primary pulmonary hypertension and discuss possible treatment options.

The prognosis for primary pulmonary hypertension is generally poor, and most people succumb to heart or lung failure within a few years of receiving their diagnoses. Specialists can, however, help patients lessen their symptoms with prescription medications and dietary supplements. An individual who has late-stage hypertension may need to receive oxygen therapy or be placed on a mechanical ventilator to prevent lung failure. Single or double lung transplants are sometimes effective at correcting the condition, but such procedures are not usually feasible. The outlook is best when patients receive early diagnoses and immediate treatment measures are taken.

WiseGeek is dedicated to providing accurate and trustworthy information. We carefully select reputable sources and employ a rigorous fact-checking process to maintain the highest standards. To learn more about our commitment to accuracy, read our editorial process.
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