Pulmonary Hypertension (PH)

Overview 

Pulmonary hypertension is a serious condition that develops when the pressure in the blood vessels leading from the heart to the lungs becomes too high.

Over time, as the arteries’ pressure increases, the blood vessels become stiff and narrow, and your heart has to work harder to pump blood, resulting in less oxygen in the blood. 

Pulmonary hypertension classes

There are four main classes of pulmonary hypertension, including:

• Class I. No symptoms and normal heart and lung activity. 
• Class II. No symptoms at rest. Symptoms, such as fatigue, shortness of breath, or chest pain present with normal activity.
• Class III. Symptoms appear when a person is physically active.
• Class IV. Symptoms appear at rest and during physical activity.

Pulmonary hypertension causes

High blood pressure in the arteries of the lungs from congenital heart disease.

• Connective tissue disease
• Coronary artery disease
• High blood pressure
• Liver disease
• Blood clots in the lung
• Chronic lung diseases
• Genetic makeup

Many people develop pulmonary hypertension in combination with diseases such as lung disease and heart disease. Heart failure is common in patients with pulmonary hypertension.

Pulmonary hypertension risk factors

While pulmonary hypertension can affect people of all ages, races, and ethnicities, certain risk factors increase a person’s likelihood of developing it.

Risk factors include:

• Age. People over 75 years old are more likely to develop pulmonary hypertension.
• Family history. Having a family history of pulmonary hypertension increases your chances of developing pulmonary hypertension.
• Weight. Being overweight or obese.
• Gender. Females are more likely to develop PH.
• Blood clotting disorders or a family history of developing blood clots in the lungs.
• Exposure to asbestos.
• Genetic disorders, such as congenital heart disease.
• High altitude locations.
• Certain weight loss medications.

Pulmonary hypertension symptoms

Pulmonary hypertension symptoms are similar to the symptoms caused by asthma or chronic obstructive pulmonary disease (COPD). 

Common symptoms include:

• Fatigue.
• Fainting or lightheadedness.
• Chest pain.
• Heart palpitations.
• Swelling.
• Shortness of breath.

Pulmonary hypertension complications

If left untreated, pulmonary hypertension can cause severe complications such as:

• Heart failure or heart enlargement.
• Blood clots.
• Mild to life-threatening arrhythmias.
• Pregnancy complications.
• Life-threatening bleeding in the lungs.

Pulmonary hypertension diagnosis

Pulmonary hypertension is challenging to diagnose in a routine physical exam. As the condition progresses, the symptoms of pulmonary hypertension are similar to other heart and lung conditions.

If your doctor suspects you have pulmonary hypertension, he or she will perform a physical exam, take a full medical history, and order diagnostic tests. During a clinic visit, your doctor will ask you what symptoms you have and determine if you have a family history of PH.

Pulmonary hypertension tests

• Blood tests. A blood test can help determine what is causing your pulmonary hypertension and reveal any related complications.
• Chest X-ray. A chest X-ray can show if you have an enlarged right ventricle or pulmonary arteries and show signs from other heart or lung conditions.
• Electrocardiogram (ECG). An electrocardiogram can detect heart arrhythmias and right ventricle strain or enlargement.
• Echocardiogram (Echo). An Echo can help determine the right ventricle’s size and thickness and measure the pulmonary arteries’ pressure.
• Magnetic resonance imaging (MRI). An MRI can show how well the right ventricle functions and how the blood flows in the arteries that go to the lungs.
• Pulmonary function test (PFT). A PFT measures how much air your lungs can hold.
• Open-lung biopsy. An open lung biopsy is a surgery that can determine the cause of pulmonary hypertension.

Pulmonary hypertension treatment

There is not a cure for pulmonary hypertension. The goal of treatment is to manage your symptoms and improve your quality of life. Your provider will develop a customized treatment plan for your case. 

Treatments include:

• Inhaled medication. Oral, inhaled, and intravenous (IV)/subcutaneous medications can help relieve symptoms associated with PH.
• Diuretics to reduce swelling in the feet.
• Anticoagulants to prevent blood clots.
• Pulmonary rehabilitation and daily exercise can improve breathing and your quality of life. 
• Oxygen therapy. Using a portable oxygen tank can help patients breathe better.

When to seek care 

While pulmonary hypertension is difficult to diagnose in the early stages, the earlier PH is diagnosed, the more successful our care teams will be in stopping or slowing the disease’s progression. 

If left untreated, the condition will continue to worsen, potentially becoming life-threatening.