Pulmonary Hypertension

Gene mutations are found in some people who have a family history of pulmonary arterial hypertension. Mutations are also found often in patients who do not have a family history.

Many medical conditions can cause pulmonary hypertension. One type of pulmonary hypertension—called pulmonary arterial hypertension—is caused by conditions that result in narrowing of the pulmonary arteries themselves, such as scleroderma or HIV. Narrowed blood vessels can increase blood pressure in the lungs.

Medical conditions that can cause pulmonary hypertension include:

• blood clots in the lungs, called pulmonary embolism, a type of venous thromboembolism (VTE)
• Chronic exposure to high altitude
• Chronic kidney failure
• Congenital heart defects or congenital narrowing of the pulmonary arteries
• Connective tissue diseases, such as scleroderma
• HIV
• Infection with parasites, such as schistosomiasis or Echinococcus, which are tapeworms
• Left heart diseases, such as left heart failure, which may be caused by high blood pressure throughout your body or ischemic heart disease; and heart valve diseases, such as aortic stenosis and mitral valve disease
• Liver diseases, such as cirrhosis, that lead to higher-than-normal blood pressures in the liver
• Lung diseases, such as chronic obstructive pulmonary disease (COPD), interstitial lung disease, or sleep apnea
• Metabolic disorders, such as thyroid disorders or Gaucher disease
• Sarcoidosis
• Sickle cell disease
• tumor in the lungs

• Treatment will discuss medicines and procedures that your doctors may recommend if you are diagnosed with pulmonary hypertension.

Your risk of pulmonary hypertension goes up as you get older, although it may occur at any age. The condition is typically diagnosed between ages 30 and 60.

You may be at an increased risk of pulmonary hypertension if you have or are exposed to the following:

• Asbestos or silica
• Infection caused by parasites such as schistosomiasis or Echinococcus, which are tapeworms

Certain genetic disorders, such as Down syndrome, congenital heart disease, and Gaucher disease, can increase your risk of developing pulmonary hypertension.

A family history of blood clots or pulmonary embolism also increases your risk of developing pulmonary hypertension.

Unhealthy lifestyle habits can increase the risk of pulmonary hypertension. These habits include:

• Illegal drugs, such as cocaine and amphetamines
• Smoking

Some medicines may increase your risk of pulmonary hypertension, including:

• Chemotherapy medicines to treat cancer, such as dasatinib, mitomycin C, and cyclophosphamide
• Selective serotonin reuptake inhibitors (SSRIs) to treat depression and anxiety. SSRIs may cause pulmonary arterial hypertension in newborns whose mothers have taken these medicines during pregnancy.
• Weight-loss drugs such as fenfluramine and dexfenfluramine, which are no longer approved for weight loss in the United States

Certain medical conditions may increase your risk of developing pulmonary hypertension:

• Blood clotting disorders, such as blood clots in the lungs, a higher-than-normal platelet count in your blood, and conditions that make your blood more likely to clot, such as protein S and C deficiency, factor V Leiden thrombophilia, antithrombin III deficiency, and antiphospholipid syndrome
• Chronic kidney disease
• Diseases that change the structure of the chest wall, such as scoliosis
• Infections such as hepatitis B or C
• Liver disease such as cirrhosis
• Surgical removal of the spleen
• Thyroid diseases

Pulmonary hypertension is more common in women than in men. Pulmonary hypertension with certain types of heart failure is also more common in women.

Based on your symptoms or risk factors, your doctor may recommend the following tests to screen for changes in the heart or lungs that may be related to pulmonary hypertension.

• Echocardiography to look at your heart’s function and structure and estimate pulmonary artery pressure
• Electrocardiography (ECG or EKG) to look for signs of changes in your heart or abnormal rhythms in your heart’s electrical activity.
• Pulmonary function tests to look for changes in lung function for conditions such as systemic sclerosis, COPD, or interstitial lung diseases

Based on the results of these screening tests, your doctor may do follow-up tests to see whether you have higher-than-normal pressures in the pulmonary arteries. These other tests can help diagnose pulmonary hypertension.

To help prevent pulmonary hypertension, your doctor may recommend controlling certain medical conditions, avoiding certain medicines or illegal drugs, and protecting yourself against environmental hazards that are risk factors.

• Diagnosis will discuss tests and procedures that your doctor may use to diagnose pulmonary hypertension.
• Living With will discuss what your doctor may recommend to prevent your pulmonary hypertension from recurring, getting worse, or causing complications.
• Research for Your Health will discuss how we are using current research and advancing research to prevent pulmonary hypertension.
• Participate in NHLBI Clinical Trials will discuss our open and enrolling clinical studies that are investigating prevention strategies for pulmonary hypertension.

Signs and symptoms of pulmonary hypertension may include the following:

• Chest pain
• Cough that is dry or may produce blood
• Fatigue
• Hoarseness
• Light-headedness, fainting, or dizziness
• Nausea and vomiting
• Shortness of breath, first with physical activity and then without it as the disease gets worse
• Swelling of your abdomen, legs, or feet caused by fluid buildup
• Weakness
• Wheezing

Complications of pulmonary hypertension may include the following:

• Anemia
• Arrhythmias and bundle branch blocks of the heart
• Blood clots in the pulmonary arteries
• Bleeding in the lungs, which may be life-threatening
• Heart failure, especially right ventricular failure
• Liver damage from increased pressure in the right heart
• Pericardial effusions, which is a collection of fluid in the sac-like structure around the heart
• Pregnancy complications that can be life-threatening for the mother and baby

• Diagnosis will discuss tests and procedures used to detect signs of pulmonary hypertension and help rule out other conditions that may mimic pulmonary hypertension.
• Treatment will discuss treatment-related complications or side effects.

Normal pressure in the pulmonary arteries is between 11 and 20 millimeters of mercury (mm Hg) when measured by cardiac catheterization. Your doctor may perform the following tests to confirm high pressures in the pulmonary arteries.

• Cardiac catheterization to provide a definite diagnosis of pulmonary hypertension. A diagnosis of pulmonary hypertension is made if the pulmonary artery pressure is 25 mm Hg or greater while at rest.
• Echocardiography to estimate pulmonary artery pressure. An estimated pulmonary artery pressure of 35 to 40 mm Hg or greater on echocardiography suggests pulmonary hypertension. A diagnosis of pulmonary hypertension may be made when enough changes are seen on an echocardiogram. If the echocardiogram suggests pulmonary hypertension, then right heart catheterization may be the next step.

Your doctor may ask you about any signs and 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.

Your doctor may order blood tests and imaging tests to help diagnose pulmonary hypertension.

• Blood tests to look for increased risk of blood clots, stress on the heart, or anemia.
• Cardiac MRI to get detailed pictures of the structure and functioning of the heart and surrounding blood vessels.
• Chest X-ray to look at the size and shape of the heart and surrounding blood vessels, including the pulmonary arteries.
• Echocardiogram (echo) to look for signs of pulmonary hypertension and also study the heart’s structure and functioning. If the echocardiogram suggests pulmonary hypertension, then right heart catheterization may be the next step.
• Electrocardiogram (ECG or EKG) to look for signs of changes in your heart or abnormal rhythms in your heart’s electrical activity caused by pulmonary hypertension. You may still have pulmonary hypertension if you have a normal ECG.

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.

• Return to Risk Factors to review family history, medical conditions, or other factors that increase your risk of developing pulmonary hypertension.
• Return to Signs, Symptoms, and Complications to review common signs and symptoms of pulmonary hypertension.

Depending on the cause of your pulmonary hypertension, your doctor may recommend healthy lifestyle changes.

• Heart-healthy eating, which 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 as recommended and supervised by your doctor
• Physical rehabilitation to improve your ability to exercise and also boost your quality of life

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.

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 atria 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.

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

• Research for Your Health will discuss how we are using current research and advancing research to treat people with pulmonary hypertension.
• Participate in NHLBI Clinical Trials will discuss our open and enrolling clinical studies that are investigating treatments for pulmonary hypertension.
• Living With will discuss what your doctor may recommend, including lifelong lifestyle changes and medical care to prevent your condition from recurring, getting worse, or causing complications.

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.

Return to Treatment to review possible treatment options for your pulmonary hypertension.

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
• Pulmonary 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.

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 ischemic 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.

Even with treatment, pulmonary hypertension may lead to serious complications such as heart failure and arrhythmias. Know the signs and symptoms of pulmonary hypertension and how to recognize the possible complications.

If you are taking a blood thinner, this will increase your risk of bleeding. If you experience any abnormal bleeding, such as blood in your stool, black stool, or coughing up blood, contact your doctor right away. If you fall while taking a blood thinner, you are at higher risk for bleeding inside your head. Let your doctor know if you have fallen while taking a blood thinner.

Some treatments for pulmonary hypertension must be given through a long-term intravenous (IV) line. Call your doctor right away if you have any signs of infection. Signs of infection include redness, swelling, or yellow discharge where the IV is inserted; a fever of 100.3°F or higher; and chills.