Definition
Pulmonary hypertension occurs when the pressure in the blood vessels that carry blood from your heart to your lungs is higher than normal. One type of pulmonary hypertension is pulmonary arterial hypertension (PAH).
Pulmonary hypertension can happen on its own or be caused by another disease or condition. In the United States, the most common cause of pulmonary hypertension is left heart disease, such as left heart failure. There are several other medical conditions and environmental factors that can increase the risk of developing pulmonary hypertension.
If you have pulmonary hypertension, your heart has to work harder to pump blood into the lungs. This increase in pressure can damage your heart. It can also cause other symptoms such as shortness of breath, chest pain, and lightheadedness. Your healthcare provider will consider your symptoms and health history before conducting tests to diagnose pulmonary hypertension.
Treatments for pulmonary hypertension will depend on the cause of the condition. Many times, there is no cure for pulmonary hypertension, but your healthcare provider can work with you to manage the symptoms. This may include medicine or healthy lifestyle changes.
Left heart disease and chronic pulmonary diseases are well-recognized risk factors for pulmonary hypertension and are prevalent in minorities, including the Black community.
Symptoms of pulmonary hypertension are sometimes hard to recognize. People may have symptoms for years before being diagnosed. This is because many symptoms of pulmonary hypertension are also symptoms of other medical conditions.
Symptoms
Some symptoms of pulmonary hypertension include:
- Chest pain
- Coughing that is dry or produces blood
- Shortness of breath
- Dizziness that may lead to fainting
- Nausea and vomiting
- Hoarseness
- Swelling of the abdomen, legs, or feet
- Weakness
- Wheezing, which is a whistling sound when you breathe out
Symptoms can get worse over time. For example, in the early stages of pulmonary hypertension, you may only have shortness of breath with exercise. As the disease progresses, shortness of breath will occur more often.
When to call 9-1-1
If you experience chest pain and shortness of breath, seek emergency medical care. This may be a sign of a heart attack or a blood clot in your lungs (pulmonary embolism).
Diagnosis
To diagnose pulmonary hypertension, your doctor may ask you questions about your medical history and do a physical exam. Based on your symptoms and risk factors, your doctor may refer you to a lung specialist (pulmonologist) or a heart and blood vessel specialist (cardiologist). Your doctor will diagnose you with pulmonary hypertension if tests show higher-than-normal pressure in the arteries of the lungs (pulmonary arteries).
Medical history and physical exam
Your doctor may ask you about any symptoms you have been experiencing and any risk factors such as other medical conditions you have.
Your doctor will also perform a physical exam to look for signs that may help diagnose your condition. As part of this exam, your doctor may do the following:
- Check whether the oxygen levels in your blood are low. This may be done by pulse oximetry, in which a probe is placed on your finger to check your oxygen levels.
- Feel your liver to see if it is larger than normal.
- Listen to your heart to see if there are changes in how it sounds, and also to find out if your heartbeat is faster than normal or irregular or if you have a new heart murmur.
- Listen to your lungs for sounds that could be caused by heart failure or interstitial lung disease.
- Look at the veins in your neck to see if they are larger than normal.
- Look for swelling in your abdomen and legs that may be caused by fluid buildup.
- Measure your blood pressure.
Diagnostic tests
There are many tests that doctors can use to tell if you have pulmonary hypertension.
The most common tests to measure the pressure in your pulmonary arteries are cardiac catheterization and echocardiography. Normal pressure in the pulmonary arteries is between 11 and 20 millimeters of mercury (mm Hg). If the pressure is too high, you may have pulmonary hypertension. A pressure of 25 mm Hg or greater measured by cardiac catheterization or 35 to 40 mm Hg or greater on echocardiography suggests pulmonary hypertension.
Other tests may include:
- Blood tests look for blood clots, stress on the heart, or anemia.
- Heart imaging tests, such as cardiac MRI, take detailed pictures of the structure and functioning of the heart and surrounding blood vessels.
- Lung imaging tests, such as chest X-ray, looks at the size and shape of the heart and surrounding blood vessels, including the pulmonary arteries.
- Electrocardiogram (ECG or EKG) looks for changes in the electrical activity of your heart. This can help detect if certain parts of the heart are damaged or working too hard. In pulmonary hypertension, the heart can become overworked due to damage or changes in the pulmonary arteries.
Test for other medical conditions
Your doctor may order additional tests to see whether another condition or medicine may be causing your pulmonary hypertension. Doctors can use this information to develop your treatment plan.
Causes
The cause of pulmonary hypertension is not always clear. Certain medical conditions can damage, change, or block the blood vessels of the pulmonary arteries, which can lead to pulmonary hypertension.
Some examples of medical conditions include:
- Left heart diseases, such as left heart failure, which may be caused by high blood pressure throughout your body or coronary heart disease
- Other heart and blood vessel diseases such as congenital (inherited) heart defects
- Lung diseases such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, emphysema, or sleep apnea
- Other medical conditions such as liver disease, sickle cell disease, blood clots in the lungs, or connective tissue disorders like scleroderma
Risk Factors
Several factors can increase your risk of developing pulmonary hypertension:
- Age: Pulmonary hypertension can occur at any age, but your risk increases as you get older. The condition is usually diagnosed between ages 30 and 60.
- Environment: You may be at an increased risk of pulmonary hypertension if you have or are exposed to Asbestos or certain infections caused by parasites.
- Family history and genetics: Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can increase your risk of pulmonary hypertension. A family history of blood clots also increases your risk.
- Lifestyle habits: Unhealthy lifestyle habits such as smoking and illegal drug use can raise your risk of developing pulmonary hypertension.
- Medicine: Some prescribed medicines used to treat cancer and depression may increase your risk of pulmonary hypertension.
- Sex: Pulmonary hypertension is more common in women than in men. Pulmonary hypertension with certain types of heart failure is also more common in women.
Complications
To understand pulmonary hypertension, it is helpful to understand the job of the heart and lungs, and the flow of blood.
As your heart beats, it sends blood throughout the body. The tissues of the body need oxygen from the blood to function properly. After the tissues use oxygen from the blood, they send oxygen-poor blood to the right side of the heart. Then, the heart pumps oxygen-poor blood through the pulmonary arteries to the lungs so the lungs can add oxygen to the blood. The force (pressure) of the blood against the walls of the pulmonary arteries is called the pulmonary pressure.
When this pressure gets too high, the pulmonary arteries become narrow or blocked. This makes the blood not flow as well, so the heart has to work harder to maintain blood flow into the lungs. Over time, this can cause damage to the heart and lungs.
Pulmonary hypertension can get worse over time and lead to serious problems, including:
- Anemia, which can cause your body to not get enough oxygen-rich blood
- Arrhythmias, which are problems with the speed of your heartbeat
- Blood clots in the pulmonary arteries.
- Bleeding in the lungs
- Heart failure
- Liver damage
- Pericardial , which is a collection of fluid around the heart
- Pregnancy complications that can be life-threatening for the mother and baby
To help prevent some of the complications of pulmonary hypertension, your doctor may recommend the following.
- Make heart-healthy lifestyle changes such as heart-healthy eating if your pulmonary hypertension is due to heart failure from heart disease or high blood pressure.
- Engage in regular physical activity. Before starting any exercise program, ask your doctor about what level of physical activity is right for you.
- Avoid high altitudes when possible and discuss with your doctor any plans for air travel or visits to places at high altitude.
- Talk to your doctor if you are planning to get pregnant, as there is an increased risk of pregnancy complications.
- Treat other medical conditions, such as COPD, heart conditions, and sleep apnea.
Treatment
If you are diagnosed with pulmonary hypertension, your doctor will determine your treatment plan based on the cause of disease, if it is known. There is usually no cure for pulmonary hypertension, but there are ways to keep your symptoms from getting worse. Your doctor will recommend the best management plan for you.
Healthy lifestyle changes
Depending on the cause of your pulmonary hypertension, your doctor may recommend healthy lifestyle changes:
- Heart-healthy eating includes eating less salt, to lower blood pressure or cholesterol if high levels of these contributed to the cause of your pulmonary hypertension. Eating less salt will help control your body fluids and may improve heart function.
- Physical activity that may be supervised through an exercise program such as pulmonary rehabilitation.
Medicine
Medicines to treat pulmonary hypertension may include:
- Anticoagulation or blood thinners to prevent blood clots in people whose pulmonary hypertension is caused by chronic blood clots in the lungs. These thinners also can help some people who have pulmonary arterial hypertension, heart failure, or other risk factors for blood clots.
- Digitalis or digoxin to control the rate blood is pumped throughout the body.
- Vasodilator therapy to relax blood vessels and lower blood pressure in the pulmonary artery most affected in people who have pulmonary arterial hypertension. This includes calcium channel blockers such as nifedipine and diltiazem, as well as newer groups of medicines called endothelin receptor antagonists and phosphodiesterase type 5 inhibitors.
Procedures and therapies
Your doctor may recommend a procedure, surgery, or therapy to treat pulmonary hypertension:
- Oxygen therapy if oxygen levels in the blood are too low.
- Balloon atrial septostomy to decrease pressure in the right heart chambers and improve the output of the left heart and oxygenation of the blood. In this procedure, a small hole is made in the wall between the right and left to allow blood to flow from the right to the left atrium.
- Balloon pulmonary angioplasty to lower the blood pressure in your pulmonary artery and improve heart function in people who cannot have a pulmonary endarterectomy.
- Pulmonary endarterectomy surgery to remove blood clots from the inside of the blood vessels of the lungs.
Treatments for other conditions
Your doctor may recommend medicines or procedures to treat the condition that is causing your pulmonary hypertension.
- Blood pressure medicines such as angiotensin-converting enzymes inhibitors, beta-blockers, or calcium channel blockers when left heart disease is the cause
- Blood transfusions or hydroxyurea to treat sickle cell disease
- Heart valve repair
- Iron supplements to increase blood iron levels and improve anemia
Prevention
Prevention is not always possible since the cause of pulmonary hypertension is not always clear. Your doctor may suggest a preventative screening if you have a known risk factor or medical condition that causes pulmonary hypertension. Your doctor may also recommend prevention strategies to help you lower your risk of developing pulmonary hypertension. Talk to your doctor about your risk factors and concerns to learn more.
Follow-up Care
Your follow-up care may include recommendations such as these:
- Participate in support groups, counseling, and education efforts that can help you manage the activities of daily living, experience a successful pregnancy, and generally improve the quality of your life.
- Get the recommended vaccines, which often include a vaccine for pneumococcus and an influenza, or flu, shot every year at the start of flu season.
Monitoring your condition
Talk to your doctor about new or concerning symptoms. People who have pulmonary hypertension may need regular tests. Your doctor may recommend the following to monitor your condition and treatment response:
- Six-minute walk test to monitor your ability to exercise
- Blood tests to check hemoglobin, iron, and electrolyte levels; kidney, liver, and thyroid function; your blood’s ability to clot; and signs of stress on the heart
- Cardiac catheterization
- Cardiac MRI to monitor your heart’s size and how well it is working
- Chest X-ray
- Echocardiography to monitor your heart’s size and how well it is working, and measure the pressure in your right heart chambers
- Electrocardiogram to check for irregular heartbeats
- Lung function tests to check for any change in your lung function
If your pulmonary hypertension is severe or does not respond to treatment, your doctor may talk to you about a lung transplant or a heart and lung transplant.