Learn more about the warning signs and symptoms and treatment of high blood pressure in the lungs.
Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart.
In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs are narrowed, blocked or destroyed. The damage slows blood flow through the lungs, and blood pressure in the lung arteries rises. The heart must work harder to pump blood through the lungs. The extra effort eventually causes the heart muscle to become weak and fail.
In some people, pulmonary hypertension slowly gets worse and can be life-threatening. Although there's no cure for some types of pulmonary hypertension, treatment can help reduce symptoms and improve quality of life.
When blood vessels in the lungs become thickened, narrowed, blocked or destroyed, it's harder for blood to flow through the lungs. As a result, blood pressure increases in the lungs, a condition called pulmonary hypertension.
The signs and symptoms of pulmonary hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease progresses.
Pulmonary hypertension signs and symptoms include:
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 hypertension.
Pulmonary hypertension is classified into five groups, depending on the cause.
Eisenmenger syndrome is a type of congenital heart disease that causes pulmonary 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 hypertension.
A typical heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of the heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.
Pulmonary hypertension is more often diagnosed in people ages 30 to 60. Growing older can increase the risk of developing Group 1 pulmonary hypertension, called pulmonary arterial hypertension (PAH). However, PAH from an unknown cause (idiopathic PAH) is more common in younger adults.
Other things that can raise the risk of pulmonary hypertension include:
Potential complications of pulmonary hypertension include:
Right-sided heart enlargement and heart failure (cor pulmonale). In cor pulmonale, the heart's right lower chamber (ventricle) becomes enlarged. It has to pump harder than usual to move blood through narrowed or blocked pulmonary arteries.
As a result, the heart walls thicken and the right ventricle expands to increase the amount of blood it can hold. But these changes create more strain on the heart, and eventually the right ventricle fails.
Pulmonary hypertension is hard to diagnose early because it's not often detected during a routine physical exam. Even when pulmonary hypertension is more advanced, its signs and symptoms are similar to those of other heart and lung conditions.
To diagnose pulmonary hypertension, a health care provider will perform a physical exam and review your symptoms. You'll likely be asked questions about your medical and family history.
Blood and imaging tests done to help diagnose pulmonary hypertension may include:
Echocardiogram. Sound waves are used to create moving images of the beating heart. An echocardiogram shows blood flow through the heart. This test may be done to help diagnose pulmonary hypertension or to determine how well treatments are working.
Sometimes, an echocardiogram is done while exercising on a stationary bike or treadmill to learn how activity affects the heart. If you have this test, you may be asked to wear a mask that checks how well the heart and lungs use oxygen and carbon dioxide.
Right heart catheterization. If an echocardiogram reveals pulmonary hypertension, you'll likely have a right heart catheterization to confirm the diagnosis.
During this procedure, a cardiologist places a thin, flexible tube (catheter) into a blood vessel, usually in the groin. The catheter is gently guided into the right lower heart chamber (right ventricle) and pulmonary artery. A cardiologist can then measure blood pressure in the main pulmonary arteries and the right ventricle.
Other tests may be done to check the condition of the lungs and pulmonary arteries and further determine the cause of pulmonary hypertension:
Computerized tomography (CT). This imaging test takes a series of X-rays to create cross-sectional pictures of the bones, blood vessels and soft tissues inside the body. Dye (contrast) may be injected into a vein to help the blood vessels show up more clearly on the images.
A heart (cardiac) CT scan can show the size of the heart and any blockages in the pulmonary arteries. It can help diagnose lung diseases that might lead to pulmonary hypertension such as COPD or pulmonary fibrosis.
If a family member has had pulmonary hypertension, screening for genes that are linked with disease may be recommended. If you test positive, your health care provider might recommend screening other family members.
Once a diagnosis of pulmonary hypertension is confirmed, the condition is classified according to how the symptoms affect you and your ability to do everyday tasks.
Functional classifications of pulmonary hypertension fall into one of following categories:
There's no cure for pulmonary hypertension, but treatment is available to help improve signs and symptoms and slow the progress of the disease.
It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.
When pulmonary hypertension is caused by another condition, treatment is given for the underlying cause whenever possible.
Medications are available to help improve symptoms of pulmonary hypertension and to slow the progression of the disease.
Medications that may be prescribed to treat pulmonary hypertension symptoms or complications include:
Blood vessel dilators (vasodilators). This type of medicine relaxes and opens narrowed blood vessels, improving blood flow. Vasodilators may be taken by mouth, inhaled, injected or given by IV infusion. A commonly prescribed vasodilator for pulmonary hypertension is epoprostenol (Flolan, Veletri).
This drug continuously flows through an IV attached to a small pump, which is worn in a pack on the belt or shoulder. Potential side effects of epoprostenol include jaw pain, nausea, diarrhea, leg cramps, and pain and infection at the IV site.
Other types of vasodilators, including treprostinil (Tyvaso, Remodulin, Orenitram), can be inhaled, injected or taken by mouth. The drug iloprost (Ventavis) is given while breathing in through a nebulizer, a machine that vaporizes the medication.
Side effects associated with treprostinil include chest pain, often with headache and nausea, and breathlessness. Possible side effects of iloprost include headache, nausea and diarrhea.
Blood thinners (anticoagulants). Warfarin (Jantoven) may be prescribed to help prevent blood clots. Blood thinners increase the risk of bleeding, especially in those who are having surgery or an invasive procedure. If you take blood thinners, you'll need occasional blood tests to see if the medicine is working as it should. Don't stop taking blood thinners without first talking to your care provider.
Many other drugs, herbal supplements and foods can interact with warfarin. Always tell your provider about your diet and all the medications you take, including those bought without a prescription.
If medications do not help control the signs and symptoms of pulmonary hypertension, surgery may be recommended. Surgeries to treat pulmonary hypertension include:
Lung or heart-lung transplant. Sometimes, a lung or heart-lung transplant may be recommended, especially for younger people who have idiopathic pulmonary arterial hypertension.
Major risks of any type of transplantation include rejection of the transplanted organ and serious infection. Immunosuppressant drugs must be taken for life to help reduce the chance of rejection.
Although medical treatment can't cure pulmonary hypertension, it can lessen symptoms. Lifestyle changes also may help improve pulmonary hypertension. Consider these tips:
If you think that you might have pulmonary hypertension or are worried about your pulmonary hypertension risk, make an appointment with your health care provider.
While shortness of breath is one of the first symptoms of pulmonary hypertension, the symptom is also common with many other conditions, such as asthma.
Appointments can be brief. There's often a lot to discuss, so it's a good idea to be prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your provider.
For pulmonary hypertension, some basic questions to ask your health care provider include:
Don't hesitate to ask other questions during your appointment.
Your health care provider is likely to ask you many questions. Being ready to answer them might save time to go over any details you want to spend more time on. Your provider might ask:
It's never too late to make healthy lifestyle changes, such as quitting smoking, cutting down on salt and eating a healthy diet. These changes may help prevent pulmonary hypertension from getting worse.