Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterised by high blood pressure in the arteries of the lungs, that is a mean pressure more than 20mm hg at rest or 30mm hg after exercise that eventually leads to right ventricular failure.
PAH occurs 3-5 times more frequently in females than in males. It tends to affect females between the ages of 30 and 60. It is a rare disease with incidence of one to two individuals per million each year in the US.
The exact cause of PAH is unknown although it is held that approximately 15-20% of PAH patients have heritable or inherited PAH.
Other conditions that are associated with the development of PAH include connective tissue disorders, exposure to drugs like methamphetamine and cocaine; infections, including HIV, chronic liver disease and congenital heart abnormalities.
The common symptoms are breathing difficulty, fatigue, swelling of the feet, legs and abdomen, dizziness, chest pain and palpitations among others.
Because symptoms are similar to other common lung diseases, it can often be hard to diagnose PAH. Diagnosis is often a process of elimination. Blood tests are done as well as ECG, lung function tests and echocardiogram.
It is important to know how to manage the disease effectively. Lifestyle changes that PAH patients should adopt are as follows:
- Quitting smoking and avoiding second-hand smoke is one of the most important decisions.
- Though PAH may make you feel fatigued, it is important to stay active and exercise as much as possible. You may need to avoid activity such as weight lifting.
- Many doctors also suggest avoiding saturated fat, trans fat and cholesterol and limiting your salt intake.
- Pregnancy can put a strain on your body and for a patient with PAH be possibly life-threatening, so this is a subject you should discuss with your doctor prior to becoming pregnant.
- Medical/Surgical management: There are a wide range of oral treatment options. Endothelin Receptor Antagonists (ERAs) Phosphodiesterase Inhibitors (PDE 5 Inhibitors) , Prostacyclin Analogue, Selective IP Receptor Agonist, and Soluble Guanylate Cyclase (sGC) Stimulators are some of them. In addition, a number of supportive therapies like diuretics and anticoagulants are recommended. Some patients may require a portable oxygen tank. In the most severe cases, a heart-lung or lung transplant may be recommended.
According to Dr Preeti Sharma, associate director of cardiology at our hospital, many future therapies are in the pipe line for pulmonary hypertension like Vasoactive Intestinal Polypeptide, Gene Therapy, Targeting Pro-proliferative pathways etc which may provide relief to patients in future.
Views expressed above are the author’s own.
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