Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart.
In some people, pulmonary arterial hypertension slowly gets worse and can be life-threatening. Although there’s no cure for some types of pulmonary arterial hypertension, treatment can help reduce symptoms and improve quality of life.

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Pulmonary Arterial Hypertension

The typical heart has two upper chambers (atria) and two lower chambers (ventricles). Each time blood passes through the heart, the lower right chamber (right ventricle) pumps blood to the lungs through a large blood vessel (pulmonary artery).

In the lungs, the blood releases carbon dioxide and picks up oxygen. The blood typically flows easily through blood vessels in the lungs (pulmonary arteries, capillaries, and veins) to the left side of the heart.

However, changes in the cells that line the pulmonary arteries can cause the walls of the arteries to become stiff, swollen, and thick. These changes may slow down or block blood flow through the lungs, causing pulmonary arterial hypertension.

Symptoms

The signs and symptoms of pulmonary arterial hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease progresses.

Pulmonary arterial hypertension signs and symptoms include:

Shortness of breath (dyspnea), initially while exercising and eventually while at rest
Fatigue
Dizziness or fainting spells (syncope)
Chest pressure or pain
Swelling (edema) in the ankles, legs, and eventually the abdomen (ascites)
Bluish color to the lips and skin (cyanosis)
Fast pulse or pounding heartbeat (palpitations)

Eisenmenger syndrome and pulmonary arterial hypertension

Eisenmenger syndrome is a type of congenital heart disease that causes pulmonary arterial hypertension. It’s most commonly caused by a large hole in the heart between the two lower heart chambers (ventricles), called a ventricular septal defect.

This hole in the heart causes blood to flow incorrectly in the heart. Oxygen-carrying blood (red blood) mixes with oxygen-poor blood (blue blood). The blood then returns to the lungs — instead of going to the rest of the body — increasing the pressure in the pulmonary arteries and causing pulmonary arterial hypertension.