What Is Primary Pulmonary Hypertension?

Primary pulmonary hypertension (PPH), also called idiopathic pulmonary arterial hypertension, is a rare condition that causes high blood pressure in the arteries of the lungs.

In this condition, the blood vessels in the lungs become narrowed or damaged. This makes it harder for blood to flow through the lungs and forces the heart to work harder to pump blood.

The exact cause of primary pulmonary hypertension is not fully understood. In some people, inherited genetic changes may increase the risk of developing the condition.

Symptoms

Symptoms often develop slowly and may become more noticeable over time.

Common symptoms include:

• Shortness of breath during activity
• Fatigue
• Chest pain
• Dizziness or fainting
• Fast heartbeat or palpitations
• Swelling in the ankles or legs
• Dry cough
• Blue lips or skin

How Is It Diagnosed?

Healthcare providers diagnose primary pulmonary hypertension using a combination of exams, imaging, and heart and lung testing.

Tests may include:

• Chest X-ray
• Electrocardiogram (ECG)
• Echocardiogram
• Pulmonary function tests
• CT scan
• Blood tests
• Right heart catheterization

Right heart catheterization is the most accurate test for measuring pressure inside the pulmonary arteries.

How Is It Treated?

There is no cure for primary pulmonary hypertension, but treatment can help manage symptoms and slow disease progression.

Treatment may include:

• Medicines that relax blood vessels in the lungs
• Diuretics to reduce fluid buildup
• Oxygen therapy
• Blood-thinning medications in some cases
• Lifestyle changes to reduce strain on the heart and lungs

People with advanced disease may need evaluation for lung transplant or heart-lung transplant.

Regular follow-up care is important to monitor symptoms and heart function over time.