Pulmonary hypertension

Pulmonary hypertension is a serious, chronic disease in which the pressure in the lungs becomes too high. High pressure puts strain on the right side of your heart. Over time, this excess strain can weaken your heart or cause heart failure.

Pulmonary hypertension v. hypertension

Pulmonary hypertension is not the same as normal hypertension (also called high blood pressure), which affects your whole body.

Pulmonary hypertension can be treated

There is no single cure for pulmonary hypertension, but there are many ways to manage it. The goal of treatment is to control your symptoms and slow the progression of pulmonary hypertension.

Pulmonary hypertension: symptoms and complications

Symptoms

Pulmonary hypertension symptoms vary widely and often look like symptoms of other diseases. They also tend to develop slowly over years.

Symptoms may include:

  • Shortness of breath. At first, you may only feel short of breath when exercising. As the disease gets worse, you may feel short of breath even when you are at rest.
  • Chest pain or pressure.
  • Dizziness.
  • Fainting.
  • Fatigue, or a feeling of tiredness that does not go away with rest.
  • Heart palpitations, or a feeling that your heart is racing.
  • Pain in the upper right side of your abdomen (belly).
  • Swelling of your ankles or legs.
  • Blue color to lips and skin.

Early on, you may dismiss symptoms like shortness of breath and fatigue as signs you are out of shape or getting old. However, these can be serious signs of an underlying problem.

Talk to your doctor if you are experiencing any of these symptoms.

Complications

Left untreated, pulmonary hypertension can have severe, life-threatening complications. These include:

  • Heart failure due to an enlarged right ventricle (cor pulmonale). Pulmonary hypertension causes the right side of your heart to strain. Your heart responds to the strain by increasing the thickness of the muscle, but this puts further pressure on your heart, causing heart failure.
  • Irregular heartbeat (arrhythmia). Over time, pulmonary hypertension can cause your heart to beat irregularly. This can lead to heart palpitations or more serious complications, like blood clots. Some arrhythmias are life-threatening.
  • Pregnancy complications. Having pulmonary hypertension while pregnant can increase your risk of pregnancy complications and may be life-threatening for you and your baby.

Woman on rowing machine at gym

Diagnosing pulmonary hypertension

If you have symptoms of pulmonary hypertension, you should talk to your doctor. Pulmonary hypertension can be challenging to diagnose. In the early stages of the disease, your doctor may not catch the condition during your regular checkups. Even as the disease progresses, symptoms may be confused with other medical conditions. This can further delay your diagnosis. Still, pulmonary hypertension needs to be caught early to avoid complications.

Your doctor may need to order several different tests to diagnose pulmonary hypertrophy and rule out other conditions.

Diagnostic tests

  • Blood tests. Your doctor may run blood tests to check for other underlying problems, like thyroid or liver disease. Some blood tests can also be used to check for strain on the heart.
  • Chest X-ray. An x-ray uses high-energy beams to create a picture of your chest. It can be used to look for problems with the heart and to rule out other conditions.

Doctor sharing information with patient

  • Echocardiogram. This test uses sound waves to create an image of the heart’s chambers and valves. This allows your doctor to see how well each part is working and check whether your right ventricle is enlarged. You may have an echocardiogram while doing physical exercise.
  • Electrocardiogram (ECG or EKG). This test measures the electrical activity of your heart. It can detect if you have an abnormal heart rhythm or if there is anything off with the electrical activity.
  • Pulmonary function test. This test measures how much air your lungs can hold and how well you can inhale and exhale. Generally, this involves breathing into and out of a machine while following a doctor’s instructions. Based on this information, you and your doctor can better understand your treatment options.

Confirming the diagnosis

If diagnostic tests make your doctor suspect pulmonary hypertension, they may perform a cardiac catheterization procedure to confirm the diagnosis. They will insert a thin, flexible tube into a large artery in your leg or near your groin and thread it up to your pulmonary artery to directly measure your pulmonary arterial pressure. This procedure may also be done to see how you are responding to treatment.

Types and causes of pulmonary hypertension

According to the World Health Organization (WHO), there are five groups of pulmonary hypertension, depending on the underlying cause:

Group 1: Pulmonary Arterial Hypertension (PAH). This type of pulmonary hypertension happens when the pulmonary arteries become narrowed or damaged, which increases the pressure inside the arteries.

Causes of pulmonary arterial hypertension include:

  • Congenital heart disease, or heart problems that you are born with.
  • Connective tissue diseases, like scleroderma and lupus.
  • Other diseases, like HIV and chronic liver disease (cirrhosis).
  • Genetic mutations passed down from your parents (heritable pulmonary arterial hypertension).
  • Use of certain drugs, like methamphetamine and cocaine.

Sometimes, there is no known cause for pulmonary arterial hypertension. This is referred to as idiopathic pulmonary arterial hypertension.

Group 2: Pulmonary hypertension due to left heart disease. The left side of your heart pumps oxygenated blood from the lungs to the rest of the body. If there is a problem with the left side valves of muscle, your heart can’t keep up with the blood coming in from the lungs. This leads to a “backup” of blood, which increases blood pressure in the lungs.

Causes of PH due to left heart disease include:

  • Congenital problems with the valves or muscle on the left side.
  • Damage to the left side of the heart, which can sometimes be caused by a heart attack, diabetes, or untreated high blood pressure.

Group 3: Pulmonary hypertension due to lung disease. Lung diseases can prevent you from getting enough oxygen when you breathe in. To compensate, your body tightens the pulmonary arteries, increasing the blood pressure in your lungs.

Causes of PH due to lung disease include:

  • Chronic obstructive pulmonary disease (COPD), emphysema, and chronic bronchitis. These lung diseases make it hard for you to exhale effectively. They are most often caused by smoking.
  • Obstructive sleep apnea, a sleep breathing disorder that happens when the muscles in your throat relax too much and prevent you from breathing.
  • Pulmonary fibrosis, or scarring of the tissue in between the air sacs and airways of your lungs.
  • Living at high altitudes for prolonged periods.

Group 4: Pulmonary hypertension because of blood clots (chronic thromboembolic pulmonary hypertension). Blood clots (pulmonary embolisms) in the pulmonary arteries can lead to scarring and narrowing. Blood clotting disorders may put you at risk for this type of pulmonary hypertension.

Group 5: Pulmonary hypertension due to unknown causes. Pulmonary hypertension can be caused by many other conditions, including sickle cell disease, thyroid disease, and liver disease.

Man in greenhouse holding tray of seedlings

No matter what group you fall in, pulmonary hypertension is a serious, even life-threatening disease that needs long-term management.

Treatments for pulmonary hypertension

There is no single cure for pulmonary hypertension, but there are many ways to manage it. The goal of treatment is to control your symptoms and slow the progression of pulmonary hypertension. If your pulmonary hypertension is being caused by another disease, you will need to treat that underlying condition (if possible).

  • Anticoagulants (“blood thinners”). Your doctor may prescribe blood thinners to help prevent blood clots in your lungs. These medications slow down the clotting process, which may increase your risk for dangerous bleeding, especially if you have major surgery.
  • Calcium channel blockers. These medications lower the blood pressure in your lungs and the rest of your body. These medications are effective, but only a small number of pulmonary hypertension patients see their condition improve while on them.
  • Diuretics (water pills). These medications prevent water retention by helping your kidneys remove excess fluid. This can reduce the amount of work your heart has to do and treat symptoms like swelling.
  • Endothelin receptor antagonists. Endothelin is a chemical that contributes to the narrowing of your blood vessels. By blocking this chemical, these medications help slow the progression of pulmonary hypertension. However, they can cause liver damage, so you will need regular labs to monitor your liver function.
  • Inotropic agents (Digoxin). These medications improve your heart’s ability to pump blood. It is usually prescribed for pulmonary hypertension that causes arrhythmia.
  • Sildenafil and Tadalafil. These medications are usually used to treat erectile dysfunction, but they can also help open up the blood vessels in your lungs.
  • Vasodilators. These medications work by expanding and relaxing your blood vessels to improve blood flow. There are several different ways the medication can be delivered. You may receive it intravenously, through an IV connected to a belt or pouch that you wear. You may take a pill, or you may inhale the medication. Each medication comes with different risks and side effects, so you will have to talk to your doctor about which one works right for you.
  • CPAP therapy. CPAP therapy stands for “continuous positive airway pressure” therapy. It may be used to treat pulmonary hypertension caused by obstructive sleep apnea. Your doctor will prescribe you a CPAP machine, which you wear when you go to bed. This machine is equipped with a mask, worn over your nose or mouth, and it delivers constant airflow to your throat. This keeps your throat open while you sleep, allowing you to breathe easily.
  • Oxygen therapy. Your doctor may recommend that you breathe in pure oxygen to make sure your body is getting enough oxygen. You may need to use oxygen when you are doing physical activities. If you have severe pulmonary hypertension, you may need to wear oxygen all the time.

Surgery is usually the last line of defense against pulmonary hypertension. You may be a candidate for surgery if you:

  • Do not respond well to medications.
  • Have pulmonary hypertension because of blood clots.
  • Have severe pulmonary hypertension.

Surgery for pulmonary hypertension may involve:

  • Pulmonary thromboendarterectomy. This surgery is used to remove blood clots in the lung’s arteries.
  • Atrial septostomy. This procedure involves creating a hole between the left and right sides of your heart. This relieves pressure on your right side. However, it comes with serious risks and can cause arrhythmia.
  • Lung transplantation. If you have severe pulmonary hypertension, especially idiopathic pulmonary hypertension, you may need a lung or lung and heart transplant. This involves replacing your lungs (and potentially your heart) with those of a donor. However, transplant waitlists are long, and there is a chance your body will reject the new organ. You will need to talk with your doctor about the risks of this kind of procedure.
  • Eat well. Enjoy a heart-healthy diet full of fresh fruits, vegetables, and whole grains. Limit your consumption of refined sugars and greasy foods.
  • Maintain a healthy weight. Being overweight or obese increases your risk of developing pulmonary hypertension. Try to maintain a healthy weight through diet and exercise.
  • Quit smoking. Smoking causes lung diseases like chronic bronchitis and emphysema. These can lead to pulmonary hypertension. If you smoke, consider joining a smoking cessation program. If you don’t smoke, don’t start.
  • Avoid high altitudes. High altitudes can make pulmonary hypertension worse. If you currently live at high altitudes, you may want to move to a lower altitude.
  • Discuss pregnancy and birth control with your doctor. Pulmonary hypertension can be life threatening for you and your baby if you do become pregnant. However, some hormonal contraceptives can increase your risk for blood clots. You will need to talk to your doctor about what birth control is right for you and what to do if you get pregnant.

Together, treatment and lifestyle choices can help you get back to the activities you love and make the most of every day.