Pulmonary Arterial Hypertension (PAH)

Pulmonary arterial hypertension (PAH) is a rare and serious disease that affects the blood vessels in the lungs. These vessels become too narrow or stiff, which increases the pressure in the pulmonary arteries. As a result, the right side of the heart has to work much harder to pump blood, which can weaken it over time. If left untreated, PAH can lead to right heart failure. There is another rare form called chronic thromboembolic pulmonary hypertension (CTEPH), which is caused by blood clots in the pulmonary arteries (pulmonary embolism) and can present in a similar way to PAH.

Symptoms may appear gradually. It is important to pay attention to them:

• Shortness of breath, even with moderate effort (climbing stairs, walking)
• Unusual, persistent fatigue
• Palpitations (rapid or irregular heartbeat)
• Chest pain
• Dizziness or fainting
• Swelling of the legs or ankles (edema)

These symptoms are not specific to PAH, but if they persist, consult your doctor. Be aware: swollen legs and fainting (syncope) during exertion are very serious signs that require prompt medical attention.

PAH can affect people of all ages, from early childhood to the elderly. Certain medical conditions predispose individuals to PAH and require annual screening by cardiac ultrasound—this is the case for autoimmune diseases (such as systemic sclerosis) and hereditary forms.

• Patients with congenital heart disease
• Autoimmune diseases (e.g., scleroderma, lupus)
• People living with HIV
• Associated with certain drugs or toxic substances (e.g., amphetamine derivatives, Mediator®)

Causes and risk factors

• Idiopathic: no known cause
• Genetic: hereditary form
• Associated with other diseases (e.g., heart defects, liver cirrhosis, autoimmune diseases)
• Related to certain medications or toxic substances (e.g., Mediator®, amphetamines, and some chemotherapy drugs)

Pulmonary arterial hypertension in Belgium

This is a rare disease: about 15 to 50 cases per million inhabitants. This likely represents several hundred patients, but many remain undiagnosed. Early diagnosis is essential to improve quality of life and longevity.

How is Pulmonary Arterial Hypertension diagnosed?

• Pulmonary function tests: You breathe into a device that measures your lung capacity. This helps rule out other respiratory diseases.
• Electrocardiogram (ECG)
• Echocardiography: A simple, painless test that assesses heart function and detects elevated pressure in the pulmonary arteries.
• Right heart catheterization: This is the key test to confirm PAH. A small tube is inserted into a vein to directly measure blood pressure in the pulmonary arteries. The test is done under local anesthesia, is quick, and generally well tolerated.
• Chest CT scan or cardiac MRI: These provide detailed images of the heart and lungs and can detect abnormalities such as clots or malformations.
• Exercise test
• Blood tests

What treatments are available?

At home

• Oral medications
• Vasodilators and antiproliferative agents: to widen the pulmonary vessels and alter their structure
• Diuretics: to reduce swelling and relieve the heart’s workload
• Oxygen therapy: If oxygen levels in the blood are too low, oxygen can be provided at home via a mask or nasal cannula.

H.U.B also offers advanced outpatient therapies

• Continuous infusions: Some treatments are administered continuously via a pump connected to a small catheter placed under the skin or into a central vein. These require specialized care.
• Targeted injectable therapies: Specific medications are injected regularly to relax the pulmonary arteries and improve blood flow.
• Cardiopulmonary rehabilitation: Supervised sessions of light physical activity to improve breathing during exertion, boost endurance, and manage stress and fatigue.
• Follow-up care may also include physiotherapists, nurses, psychologists, or dietitians, depending on the patient's needs.

Specialized follow-up is essential

Pulmonary Hypertension requires multidisciplinary care in a specialized center. At the Brussels University Hospital (H.U.B), the Pulmonary Hypertension & Heart Failure Clinic brings together:

• Expert cardiologists and pulmonologists
• Specialized nurses
• Physiotherapists, dietitians, psychologists
• Surgeons, geneticists, and radiologists
• A personalized and coordinated care approach

Would you like information for yourself or one of your loved ones?

Contact the Pulmonary Hypertension and Heart Failure Clinic at H.U.B

By phone at +32 (0)2 555 59 53 or by email at SecMed [dot] Cardio [dot] erasme [at] hubruxelles [dot] be (SecMed[dot]Cardio[dot]erasme[at]hubruxelles[dot]be) 

Coordination with your primary care physician is possible.