Pulmonary hypertension

What is pulmonary hypertension?

Pulmonary hypertension affects the heart’s ability to pump blood to the lungs. It may arise as a secondary complication of other heart or lung conditions. A primary disorder affecting the arteries of the lungs may also lead to pulmonary hypertension.

In pulmonary hypertension, the lung’s blood vessels become stiff, narrowed or damaged, increasing the pressure with which blood flows through them. This reduces blood flow through the lungs, which causes lower levels of oxygen in the blood.

These issues lead to high blood pressure in the pulmonary arteries (arteries in the lungs) and make the lower right ventricle (section) of the heart work harder than it should. Over time, the right ventricle enlarges and weakens (cor pulmonale). When the condition gets bad enough, the right ventricle can’t do its job, causing right heart failure. This is a potentially life-threatening condition.

When you come to Advocate Heart Institute, you’re in caring and capable hands. You’ll benefit from the expertise of the region’s most experienced heart and vascular specialists. Through our Pulmonary Heart Disease Program, you’ll receive appropriate therapies right from the start.

Pulmonary hypertension risk factors

Pulmonary hypertension is more common among women, Black people who are non-Hispanic and in people older than 75. It happens at all ages and becomes more common with aging.

Symptoms of pulmonary hypertension

Early-stage pulmonary hypertension symptoms may include:

• Shortness of breath
• Tiredness (fatigue)

As the disease progresses, pulmonary arterial hypertension symptoms may include:

• Abdominal or lower extremity swelling
• Lightheadedness, dizziness or fainting (syncope)
• Decreased appetite
• Dry cough, wheezing or hoarseness
• Elevated heart rate, even when resting (palpitations)
• Chest pain or pressure

Complications of pulmonary hypertension

Right heart failure is the most frequent complication of pulmonary arterial hypertension. When the arteries in your lungs are affected, the right side of your heart has to work harder to keep blood moving through your lungs.

When pulmonary hypertension starts to happen, the walls of your heart start to thicken and the right ventricle (lower right section of the heart) increases in size. Without treatment, these changes will eventually cause the right ventricle to fail.

Other complications, which can be life-threatening, are:

• Pericardial effusion (fluid buildup in the tissue sac surrounding your heart)
• Bleeding in the lungs
• Coughing up blood
• Blood clots in the small arteries in your lungs
• Damage to other organs including your liver and kidneys
• Pregnancy complications that can be life-threatening for mother and fetus
• Arrhythmia (irregular heartbeat)
• Syncope (fainting)

Pulmonary hypertension causes & groups

Your doctor may not be able to find a cause for your pulmonary hypertension. There are many known causes including some that are related to heart conditions, some related to other health conditions and some related to your life experiences.

According to the World Health Organization, pulmonary hypertension can be divided into five groups depending on what causes or contributes to it.

Group 1: Pulmonary arterial hypertension (PAH)

When obstruction in the small arteries of the lungs is the cause of increased pressure in the vessels. Causes include:

• Drug use
• HIV infection
• Connective tissue or autoimmune disorders (such as scleroderma)
• Unidentified causes

Group 2: Pulmonary hypertension due to left-side heart disease

Pulmonary hypertension caused by disorders in how the heart muscle or heart valves work. These problems cause blood pressure in the lungs to rise and retention of blood in the left side of the heart.

Group 3: Pulmonary hypertension caused by underlying lung diseases or hypoxemia

Diseases and conditions that cause low oxygen in your blood are:

• Chronic obstructive pulmonary disease (COPD)
• Emphysema
• Pulmonary fibrosis
• Obstructive sleep apnea
• Lung development abnormalities
• Chronic high-altitude exposure

Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH)

When blood clots in the lung’s arteries form scar-like tissue which blocks or narrows the arteries and leads to CTEPH.

Group 5: Pulmonary hypertension from numerous other disorders

When pulmonary hypertension has a cause that doesn’t fit in groups 1 through 4. This includes systemic disorders like sarcoidosis, blood disorders, metabolic disorders or tumors that obstruct pulmonary arteries.

Diagnosing pulmonary heart disease

The symptoms of pulmonary hypertension are similar to other diseases, so it can be hard to diagnose. You’ll need to see one of our specialists. They’ll use one or more tests to confirm your pulmonary hypertension diagnosis and determine whether it’s acute or chronic cor pulmonale. Find out more about heart and vascular testing and diagnosis.

Your evaluation may include:

• Cardiac catheterization: Uses thin tubes (catheters) to evaluate heart and lung pressures and function. Learn more about cardiac catheterization.
• Chest X-ray: Shows whether you have abnormally large pulmonary arteries or heart.
• Computed tomography (CT) of the chest: Allows evaluation of lung tissue for damage or scarring.
• Pulmonary function tests (PFTs): Evaluates lung function.
• Electrocardiogram (EKG): Records the heart’s electrical activity and may show signs of heart damage.
• Echocardiography: Uses sound waves to measure heart valve pressure.
• Blood testing: Measures levels of brain natriuretic peptide (BNP) in your blood. The body releases BNP when there is heart damage.
• Pulmonary ventilation/perfusion (V/Q) scan: Assesses blood and air flow to the lungs. Shows whether there’s enough oxygen in the blood or if there are blood clots in the arteries.
• CT angiogram: Uses contrast dye to highlight blood vessels and look for blood clots or signs of lung disease.
• Magnetic resonance imaging (MRI): Looks at your heart’s structure and function in more detail.
• Sleep tests (polysomnogram): Diagnoses sleep apnea, which can contribute to pulmonary hypertension.

Treating pulmonary hypertension

Pulmonary hypertension can’t be cured, but it often gets better with pulmonary hypertension management therapies to control its cause. If it’s due to worsening COPD, you may benefit from drugs that relax airways making it easier to breathe.

We use several medications to treat symptoms of pulmonary hypertension. These include medications taken by mouth, through the skin, through an IV or through inhaled therapies.

Your doctor may recommend oxygen therapy if your oxygen levels fall too low. Using an oxygen tank can help you breathe easier and feel less fatigue.

Some people with pulmonary hypertension benefit from surgeries or procedures. You may have surgery to remove blood clots from the blood vessels in your lungs.

Your doctor will work with you to determine the most effective treatment options for pulmonary hypertension for you.

Lifestyle changes

Adopting healthy habits can lower the risk of symptoms coming back after pulmonary hypertension treatment. You may be able to lower your pulmonary hypertension naturally using these preventive care habits:

• Quitting smoking
• Eating a healthy diet
• Staying physically active
• Reducing stress levels through relaxation and other techniques
• Engaging in cardiac rehab to improve fitness and make it easier to breathe

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