Pulmonary Hypertension: Causes, Symptoms, Diagnosis, Treatment

What is pulmonary hypertension?

Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the heart to the lungs become narrowed, making it difficult for blood to flow through the vessels. As a result, the blood pressure in these arteries -- called pulmonary arteries -- rises far above normal levels. This abnormally high pressure strains the right ventricle of the heart, causing it to expand in size. Overworked and enlarged, the right ventricle gradually becomes weaker and loses its ability to pump enough blood to the lungs. This could lead to the development of right heart failure.

Pulmonary hypertension occurs in individuals of all ages, races, and ethnic backgrounds, although it is much more common in young adults and is approximately twice as common in women as in men.

Why do the pulmonary arteries narrow?

Scientists believe that the process starts with injury to the layer of cells that line the small blood vessels of the lungs. This injury, which occurs for unknown reasons, may cause changes in the way these cells interact with the smooth muscle cells in the vessel wall. As a result, the smooth muscle contracts more than normal and narrows the vessel.

What are the symptoms of pulmonary hypertension?

Symptoms of pulmonary hypertension do not usually occur until the condition has progressed. The first symptom of pulmonary hypertension is usually shortness of breath with everyday activities, such as climbing stairs. Fatigue, dizziness, and fainting spells also can be symptoms. Swelling in the ankles, abdomen or legs, bluish lips and skin, and chest pain may occur as strain on the heart increases. Symptoms range in severity and a given patient may not have all of the symptoms.

In more advanced stages of the disease, even minimal activity will produce some of the symptoms. Additional symptoms include:

Eventually, it may become difficult to carry out any activities as the disease worsens.

What causes pulmonary hypertension?

The following are some known causes of pulmonary hypertension:

Pulmonary hypertension may also be caused by other conditions, and in some cases, the cause is unknown.

How is pulmonary hypertension diagnosed?

Because pulmonary hypertension may be caused by many medical conditions, a complete medical history, physical exam, and description of your symptoms are necessary to rule out other diseases and make the correct diagnosis. During the physical exam, your health care provider will:

Other tests that might be ordered include:

How is pulmonary hypertension treated?

Appropriate diagnosis and analysis of the problem is necessary before starting any treatment. Treatment varies by patient, based on the different underlying causes, but generally includes:

Listed below are medication and surgical treatment approaches.

Medications

Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depend on how severe the pulmonary hypertension is, how likely it is to progress, and a patient’s drug tolerance.

Surgical therapies

Pulmonary thromboendarterectomy: If present, blood clots in the pulmonary artery may be surgically removed to improve blood flow and lung function.

Lung transplantation: Currently, this is the only cure for pulmonary hypertension. Transplantation is reserved for advanced pulmonary hypertension that does not respond to medical therapy. The right side of the heart will generally return to normal after the lung/lungs have been transplanted. About 1,000 lung transplants are performed every year in the United States. Many people are on the waiting list, yet a shortage of donors is the major limiting factor. Your health care provider will discuss transplantation if it is an appropriate treatment option for your condition.

Heart/lung transplantation: This type of double organ transplant is very rare but is necessary for all patients who have combined lung and left heart failure.

References

 

This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. For additional health information, please contact the Center for Consumer Health Information at the Cleveland Clinic (216) 444-3771 or toll-free (800) 223-2273 extension 43771. If you prefer, you may visit www.clevelandclinic.org/health/ or www.clevelandclinicflorida.org. This document was last reviewed on: 7/15/2014

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