Pulmonary Hypertension

Pulmonary hypertension is a condition that affects the blood vessels in the lungs. It develops when the blood pressure in your lungs is higher than usual. Approximately 1% of the global population has pulmonary hypertension. 

Pulmonary hypertension makes the heart work harder than usual to pump blood into the lungs. This extra effort can damage the heart and cause symptoms such as shortness of breath, chest pain and lightheadedness.

Pulmonary hypertension can develop on its own or be caused by another disease or condition. In the United States, the most common type of pulmonary hypertension is caused by left-sided heart disease, such as left-sided heart failure.

There are five different groups of pulmonary hypertension:

• Pulmonary arterial hypertension (PAH)
• Pulmonary hypertension due to left-sided heart disease
• Pulmonary hypertension due to lung disease and/or hypoxia
• Pulmonary hypertension due to pulmonary artery obstructions, including chronic thromboembolic pulmonary hypertension (CTEPH)
• Pulmonary hypertension with unknown and/or multiple causes

The cause of pulmonary hypertension is not always clear. 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, or pressure, of the blood against the walls of the pulmonary arteries is called pulmonary pressure.

When this pressure gets too high, the pulmonary arteries become narrow or blocked. Blocked arteries result in restricted blood flow, 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.

Certain medical conditions can damage, alter or obstruct the blood vessels of the pulmonary arteries, leading to pulmonary hypertension. Some examples of medical conditions include:

• Left-sided heart diseases, such as left-sided heart failure, possibly 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

Several factors can increase your risk of developing pulmonary hypertension. Pulmonary hypertension can occur at any age, but your risk increases as you get older. The condition is typically diagnosed between the ages of 30 and 60. You may be at an increased risk of pulmonary hypertension if you have or are exposed to asbestos or certain infections caused by parasites. 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 of developing blood clots.

Unhealthy lifestyle habits such as smoking and illegal drug use can raise your risk of developing pulmonary hypertension. Some prescribed medicines used to treat cancer and depression may increase your risk of pulmonary hypertension. Pulmonary hypertension is more common in women than in men.

Preventing pulmonary hypertension is not always possible since the cause is not always clear. Your doctor may recommend a preventative screening if you have a known risk factor or a medical condition that increases your risk of developing pulmonary hypertension. Your doctor may also recommend prevention strategies to help you lower your risk of developing pulmonary hypertension. Discuss your risk factors and concerns with your doctor to learn more.

Your doctor will perform a physical exam to screen for signs that may help diagnose your condition. As part of this exam, your doctor may perform the following screening procedures:

• Check oxygen levels in your blood using pulse oximetry—a noninvasive device that measures oxygen levels by placing a probe on your finger
• Feel your liver to see if it is larger than usual
• Listen to your heart to detect any changes in its sound, and also to determine whether your heartbeat is faster than usual or irregular, or if you have developed a new heart murmur
• Listen to your lungs for sounds that may indicate heart failure or interstitial lung disease
• Examine the veins in your neck to see if they are larger than usual
• Look for swelling in your abdomen and legs that may be a result of fluid buildup
• Measure your blood pressure

Symptoms of pulmonary hypertension are sometimes hard to recognize. People may have symptoms for years before being diagnosed as having pulmonary hypertension, as symptoms can be similar to those of other medical conditions.

Symptoms can worsen over time. For example, in the early stages of this condition, you may only have shortness of breath with exercise. As the disease progresses, shortness of breath will occur more often.

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
• Fatigue
• Swelling of the abdomen, legs or feet
• Weakness
• Wheezing or a whistling sound when exhaling

To diagnose pulmonary hypertension, your doctor will conduct a thorough medical history and perform a physical exam. Based on your symptoms and risk factors, your doctor may refer you to a specialist in lung diseases (pulmonologist) or a specialist in heart and blood vessel diseases (cardiologist). Your doctor will diagnose you with pulmonary hypertension if tests show higher-than-normal pressure in the arteries of the lungs, specifically the pulmonary arteries.

Your doctor may ask you about any symptoms you have been experiencing and any risk factors, such as other medical conditions you have. They will also perform tests to determine whether you have pulmonary hypertension, including cardiac catheterization and echocardiography.

Other tests may include:

• Blood tests to check for blood clots, stress on the heart or anemia
• Heart imaging tests, such as cardiac magnetic resonance imaging (MRI) scans, for detailed images of the heart’s structure and function, as well as the surrounding blood vessels
• Lung imaging tests, such as chest X-rays, to examine the size and shape of the heart and surrounding blood vessels, including the pulmonary arteries
• An electrocardiogram (ECG or EKG) to detect changes in the electrical activity of the 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.

If you are diagnosed with pulmonary hypertension, your doctor will determine your treatment plan based on the cause of the 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 tailored to your specific needs.

Depending on the cause of your pulmonary hypertension, your doctor may:

• Recommend healthy lifestyle changes, including a heart-healthy diet that limits salt intake to lower blood pressure or cholesterol levels if high levels contribute to the cause of your pulmonary hypertension. Eating less salt can help regulate your body’s fluid balance and may improve heart function.

• Suggest supervised physical activity through an exercise program, such as pulmonary rehabilitation

  Your doctor may also recommend medication to treat pulmonary hypertension. Common prescriptions 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 can also help some people who have pulmonary arterial hypertension, heart failure or other risk factors for blood clots.
• Digitalis or digoxin to control the rate at which blood is pumped throughout the body
• Vasodilator therapy to relax blood vessels and lower blood pressure in the pulmonary arteries, which are most affected in people with pulmonary arterial hypertension. This includes calcium channel blockers, such as nifedipine and diltiazem, as well as newer groups of medications, including endothelin receptor antagonists and phosphodiesterase type 5 inhibitors.

Your care team may recommend procedures, surgery or other therapy to treat pulmonary hypertension, including:

• Oxygen therapy in cases where blood oxygen levels 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 created in the wall between the right and left atria to allow blood to flow from the right atrium to the left atrium.
• Balloon pulmonary angioplasty to lower blood pressure in the pulmonary artery and improve heart function in individuals who are not candidates for a pulmonary endarterectomy
• Pulmonary endarterectomy surgery to remove blood clots from the inside of the blood vessels of the lungs

Your doctor may recommend medications or procedures to treat the condition causing your pulmonary hypertension. These may include blood pressure medications such as angiotensin-converting enzyme inhibitors, beta blockers or calcium channel blockers when left-sided heart disease is the cause. Some cases may benefit from blood transfusions, hydroxyurea to treat sickle cell disease or heart valve repair, as well as iron supplements to increase blood iron levels and alleviate anemia.

After you are diagnosed with pulmonary hypertension, it is essential to follow up with your treatment plan, get regular care and learn how to monitor your condition. Taking these steps can slow down the progression of the disease and may improve your condition. Your specific treatment plan will depend on the cause of your pulmonary hypertension, as well as how advanced it is.

Pulmonary hypertension can get worse over time and lead to serious problems, including:

• Anemia (can cause your body not to get enough oxygen-rich blood)
• Arrhythmias, or problems with the speed of your heartbeat
• Bleeding in the lungs
• Blood clots in the pulmonary arteries
• Heart failure
• Liver damage
• Pericardial effusion, which is a collection of fluid around the heart
• Pregnancy complications, which can be life-threatening for the mother and baby