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Pulmonary hypertension

Last updated: Thursday, June 23, 2011

Pulmonary hypertension is a serious condition that causes the blood pressure in the arteries to your lungs to become abnormally high which can result in heart failure.

Pulmonary hypertension (PH) is high blood pressure in the arteries to your lungs. It is a serious condition for which there are treatments but no cure. If you have it, the blood vessels that carry oxygen-poor blood from your heart to your lungs become hard and narrow. Your heart has to work harder to pump the blood through. Over time, your heart weakens and cannot do its job and you can develop heart failure.

Overview

The World Health Organisation divides PH into five groups, based on its causes. In all groups, the average pressure in the pulmonary arteries is higher than 25 mmHg at rest or 30 mmHg during physical activity. The pressure in normal pulmonary arteries is 8–20 mmHg at rest. (The mmHg is millimeters of mercury - the units used to measure blood pressure.)

To understand PH, it helps to understand how your heart and lungs work. Your heart has two sides, separated by an inner wall called the septum.

Each side of your heart has an upper and lower chamber. The lower right chamber of your heart, the right ventricle, pumps blood to your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen.

The upper left chamber of your heart, the left atrium, receives the oxygen-rich blood from your lungs. The blood is then pumped into the lower left chamber of your heart, the left ventricle. From the left ventricle, the blood is pumped to the rest of your body through an artery called the aorta.

PH begins with inflammation and changes in the cells that line your pulmonary arteries. Other factors also can affect the pulmonary arteries and cause PH. For example, the condition may develop if:

The walls of the arteries tighten
The walls of the arteries are stiff at birth or become stiff from an overgrowth of cells
Blood clots form in the arteries

These changes make it hard for your heart to push blood through your pulmonary arteries and into your lungs. As a result, the pressure in your arteries rises. Also, because your heart is working harder than normal, your right ventricle becomes strained and weak.

Your heart may become so weak that it can't pump enough blood to your lungs. This causes heart failure - the most common cause of death in people with PH.

Signs and symptoms

Signs and symptoms of pulmonary hypertension (PH) may include:

Shortness of breath during routine activity, such as climbing two flights of stairs
Tiredness
Chest pain
A racing heartbeat
Pain on the upper right side of the abdomen
Decreased appetite

As PH worsens, you may find it hard to do any physical activities. At this point, other signs and symptoms may include:

Feeling light-headed, especially during physical activity
Fainting at times
Swelling in your legs and ankles
A bluish colour on your lips and skin

Who is at risk for PH?

PH is usually caused by other diseases or conditions, such as heart and lung diseases or blood clots. However, in some cases people inherit the condition and in others the cause is simply unknown.

Group 1 pulmonary arterial hypertension (PAH) without a known cause is rare. It affects women more often than men. People who have group 1 PAH tend to be overweight.

PH that occurs with another disease or condition is more common.

PH usually develops between the ages of 20 and 60, but it can occur at any age.

People who are at increased risk for PH include:

Those who have a family history of the condition.
Those who have certain diseases or conditions, such as heart and lung diseases, liver disease, HIV infection, or blood clots in the pulmonary arteries.
Those who use street drugs (such as cocaine) or certain diet medicines.
Those who live at high altitudes.

Diagnosis

Your doctor will diagnose PH based on your medical and family histories, a physical exam, and the results from tests and procedures.

PH can develop slowly. In fact, you may have it for years and not know it. This is because the condition has no early symptoms. When symptoms do occur, they're often like those of other heart and lung conditions, such as asthma. This makes PH hard to diagnose.

Treatment

PH has no cure. However, research for new treatments is ongoing, offering hope for the future. The earlier PH is treated, the easier it is to control. Treatments include medicines, procedures, and other therapies. These treatments can relieve PH symptoms and slow the progress of the disease.

Work with your doctor to manage your symptoms and slow the progress of PH. Get ongoing care and follow your treatment plan as your doctor advises. Call your doctor if your symptoms worsen or change.

Lifestyle changes, such as following a healthy diet, quitting smoking, and being physically active, also can help control symptoms.

(Source: National Heart, Lung and Blood Institute/National Institutes of Health)

For more information and support, contact the Pulmonary Hypertension Association of South Africa (PHSA). You can also visit the international PH Association's website. Check out their informative video below: