Pulmonary Hypertension

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. Other conditions that can cause pulmonary hypertension include sickle cell disease; pulmonary embolus, which is a type of venous thromboembolism; and chronic obstructive pulmonary disease (COPD).

The increased pressure in the blood vessels of the lungs means that your heart has to work harder to pump blood into the lungs. This can cause symptoms such as shortness of breath, chest pain, and lightheadedness. If left untreated, the increased pressure can damage your heart. This may lead to serious or life-threatening complications, such as heart failure or arrhythmias, which are irregular heart rhythms.

Why Choose Cooper for Pulmonary Hypertension Diagnosis and Treatment

Cooper provides the most comprehensive pulmonary care in southern New Jersey, providing world-class care — in your neighborhood. Our physicians are experts in the treatment and leaders in research. Utilizing the latest technology available to pinpoint and treat all types of lung and chest problems, we produce superior outcomes for our patients.

  • Nationally and internationally renowned clinical and interventional pulmonologists
  • The Cooper Breathing Center, a one-stop multidisciplinary resource for accurate, timely diagnosis and treatment planning
  • The most advanced minimally invasive techniques
  • State-of-the-art diagnostic capabilities
  • Academic health system with the largest  teaching program in South Jersey
  • Individualized care designed to meet your needs

Causes of Pulmonary Hypertension

Your genes or other medical conditions can cause pulmonary hypertension. Certain medical conditions can damage, change, or block the blood vessels of the pulmonary arteries. The cause of pulmonary hypertension is not always clear.

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
  • Tumors in the lungs

Risk Factors for Pulmonary Hypertension

You may have an increased risk for pulmonary hypertension because of your age, environment, family history and genetics, lifestyle habits, medicines you are taking, other medical conditions, or sex.

Age: 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.

Environment: 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

Family history and genetics: 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

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.

Signs, Symptoms and Complications

Signs and symptoms of pulmonary hypertension are sometimes hard to recognize, because they are similar to those of other medical conditions. People may have symptoms for years before being diagnosed with pulmonary hypertension. These symptoms may get worse over time and could eventually lead to serious complications such as right heart failure.

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 effusion, 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

Diagnosing Pulmonary Hypertension

To diagnose pulmonary hypertension, your doctor may ask you questions about your medical history and do a physical exam. Your doctor may also test you for pulmonary hypertension based on your signs and symptoms and risk factors. A diagnosis of pulmonary hypertension will be made if tests show higher-than-normal pressure in the pulmonary arteries.

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 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.

Treatment for Pulmonary Hypertension

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 no cure for pulmonary hypertension unless chronic blood clots in the lungs are the cause, so good disease management is the key.

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

Make an Appointment With a Pulmonary Hypertension Specialist at Cooper

To learn more about treatments for pulmonary hypertension or to make an appointment with a specialist, call 800.8.COOPER (800.826.6737).