What Is Pulmonary Arterial Hypertension (PAH)?

Pulmonary Arterial Hypertension (PAH) is a rare form of pulmonary hypertension where the blood vessels in your lungs are narrowed, blocked or destroyed. The damage slows blood flow through your lungs, and blood pressure in the lung arteries rises. Your heart must work harder to pump blood through your lungs.

Causes

The exact cause is unknown. It is believed that PAH occurs when there is injury to the cells that line blood vessels in the lung. When PAH occurs with no other disease, it is called Idiopathic PAH. Symptoms are often difficult to identify at first. Shortness of breath or trouble breathing may be a first indication of the condition.

The following health conditions may be causes of PAH:

• Autoimmune diseases like Lupus, rheumatoid arthritis, etc.
• Liver disease, such as cirrhosis.
• Sleep apnea or disrupted breathing during sleep.
• HIV/Human Immunodeficiency Virus.
• Family history (genetic mutation).
• Exposure to toxins (asbestos)
• Methamphetamine or cocaine.
• Heart defects at birth.

Symptoms

Some Pulmonary Arterial Hypertension (PAH) symptoms can be missed for a long time. Other symptoms are more noticeable and serious. For many people, the first indication of a potential health issue is that they feel more exhausted from their typical daily activities.

The following is a list of common pulmonary arterial hypertension signs and symptoms:

• Shortness of breath, especially when exerting yourself, as in exercise.
• Fatigue, such as increasing tiredness even during typical daily activities.
• Fluid accumulation in your stomach/abdomen.
• Swelling, usually in your feet, legs, abdomen, and neck.
• Coughing that is out of the ordinary for you.
• Dizziness, lightheadedness, or fainting.
• Chest discomfort or pain.
• Heart palpitations (racing or pounding).
• Lips and fingers turning blue.

Since Pulmonary Arterial Hypertension is a potentially life-threatening condition, we recommend immediate medical attention if you or a loved one experience any symptoms associated with the disease.

Diagnosis

An interdisciplinary medical team often diagnoses Pulmonary Arterial Hypertension. Your doctor can conduct several tests to diagnose PAH.

Tests that can help your doctor make a Pulmonary Arterial Hypertension diagnosis include:

• Echocardiogram: An ultrasound of the heart to check the size and motion of the chambers of the heart and valves.
• Cardiac catheterization: A long thin tube (catheter) is inserted into your vein to reach and repair your heart.
• Pulmonary angiogram: This is an X-ray of your blood vessels.
• Computed Tomography (CT) scan: Combines X-rays with specialized computer technology to create detailed images of the inside of your chest.
• Magnetic Resonance Imaging (MRI): A MRI uses both a magnetic field and controlled radio waves to generate clear images of your heart and blood vessels.
• Pulmonary function tests: These tests measure how well your lungs work.
• Cardiopulmonary exercise stress test: This test measures air flow in and out of your lungs when you exercise.
• Right Heart Catheterization: This test measures the main blood vessels in the heart and how much blood per minute the heart pumps to see how well the heart is working.
• Blood Tests: HIV, thyroid tests, autoimmune panels, liver tests and blood chemistry tests.

Treatment

There is no cure for PAH. However, there are medications and procedures that can slow the disease and improve quality of life for anyone suffering from this condition. The prognosis for pulmonary arterial hypertension improves with early diagnosis and treatment. The treatment will depend on the severity of the condition, your medical history, general health, and lifestyle habits, such as diet and exercise.

Pulmonary Arterial Hypertension treatment options include:

• Pills
• Inhalers
• IV drugs
• Lung transplant
• Atrial septostomy

In Atrial septostomy, a surgeon creates an incision between the left and right sides of the heart. This surgery is usually reserved for more severe cases of PAH.