Symptomatic pulmonary arterial hypertension (PAH)

Symptomatic Pulmonary Arterial Hypertension (PAH): Causes, Symptoms and Treatments

Pulmonary arterial hypertension (PAH) is a rare and serious disorder that induces an increase in the pressure in the blood vessels of the lungs. This syndrome can lead to severe disability or even death if left untreated.

PAH is caused by an imbalance between blood supply and demand in the lungs and affects the way blood vessels expand and contract. This affects blood flow in the lungs, which means that the heart has to work harder to compensate. Eventually, the heart becomes overloaded and this can lead to symptoms of PAH.

Causes of PAH

The exact cause of PAH is unknown. However, some potential factors that could lead to the development of PAH include genetics, environmental factors such as toxic exposures, and some medical conditions such as connective tissue diseases, HIV/AIDS and sickle cell disease.

Symptoms of PAH

Symptoms of PAH typically develop slowly and can include:

• Shortness of breath during activity
• Fatigue and weakness
• Chest pain or pressure
• Dizziness or fainting
• Rapid weight loss or gain
• Swelling in the ankles, feet, and legs

It’s important to note that many of these symptoms can be attributed to other medical conditions and it’s important to seek medical attention as soon as possible if you experience any of these symptoms.

Treatments for PAH

Treatment for PAH usually involves medications such as:

• Calcium Channel Blockers (CCBs) to widen the blood vessels in the lungs
• Prostacyclins to improve blood flow
• Endothelin Receptor Antagonists (ERAs) to reduce the pressure in the pulmonary arteries
• Phosphodiesterase Inhibitors (PDEs) to improve blood flow

In some cases, patients may need surgical procedures such as a heart or lung transplant to improve their symptoms. In severe cases, a ventricular assist device may be used to support the heart.

It’s important to maintain a healthy lifestyle, including regular exercise, a balanced diet, and adequate rest in order to help manage the symptoms of PAH.