The signs and symptoms of pulmonary hypertension are subtle in the early stages of the disease and may not be noticeable for months or even years. As the disease progresses, signs and symptoms become worse.
Shortness of breath (dyspnea), either while exercising or at rest
Dizziness or fainting spells (syncope)
Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)
Chest pressure or pain
Racing pulse or heart palpitations
Bluish color to your lips and skin (cyanosis)
You’ve probably heard of high blood pressure (hypertension), a common condition that affects the way your blood flows through the arteries in your body from the left side of your heart. A less common type of high blood pressure, called pulmonary hypertension, affects only the arteries in the lungs and the right side of your heart.
Pulmonary hypertension begins when tiny arteries in your lungs, called pulmonary arteries and capillaries, become narrowed, blocked or destroyed. This makes it harder for blood to flow through your lungs, which raises pressure within the pulmonary arteries. As the pressure builds, your heart’s lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing your heart muscle to weaken and sometimes fail completely.
Pulmonary hypertension is a serious illness that becomes progressively worse and is sometimes fatal. Although it isn’t curable, treatments are available that can help lessen symptoms and improve your quality of life if you have pulmonary hypertension.
Your heart has two upper and two lower chambers. Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery). In your lungs, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then flows through the pulmonary veins to the left side of your heart. From there, it’s pumped by the left ventricle to the rest of your body through another large blood vessel, the aorta.
Ordinarily, the blood flows easily through the vessels in your lungs, so your blood pressure is usually much lower in your lungs. With pulmonary hypertension, the rise in blood pressure is the end result of a process that begins with changes in the cells that line your lungs’ arteries. These changes cause the formation of extra tissue that eventually narrows or completely blocks the blood vessels. Scarring (fibrosis) usually also occurs, making the arteries stiff and narrow. This makes it harder for blood to flow, raising the pressure in the pulmonary arteries.
Secondary pulmonary hypertensionPulmonary hypertension resulting directly from another medical problem is called secondary pulmonary hypertension. This type of pulmonary hypertension is more common than idiopathic pulmonary hypertension. Medical conditions that may lead to secondary pulmonary hypertension include:
Blood clots in the lungs (pulmonary emboli)
Connective tissue disorders, such as scleroderma
Chronic obstructive pulmonary diseases, such as emphysema
Sickle cell anemia
Congenital heart disease
Chronic liver disease (cirrhosis)
Left-sided heart failure
Lung diseases such as pulmonary fibrosis, a condition that causes scarring in the tissue between the lungs’ air sacs (interstitium)
If you live above an altitude of 8,000 feet you can develop pulmonary hypertension as a result of low blood oxygen (hypoxemia) or pulmonary edema (a condition in which the air sacs in the lungs fill with fluid instead of air), which constricts the small pulmonary arteries. People who climb to high elevations without first becoming acclimated are especially at risk of developing a temporary form of pulmonary hypertension known as transient reversible pulmonary hypertension.