Pulmonary arterial hypertension (PAH) is a special form of pulmonary hypertension. PAH affects the blood vessels in the lungs, which become narrow or are destroyed. This slows the flow of blood through the lungs and increases the blood pressure in the lung arteries. To compensate for this sluggish flow of blood, the heart is forced to work harder to pump blood through the lungs. This extra effort eventually causes the heart muscles to become weak and fail.

PAH cannot be cured, but the disease can be managed through one of the treatment methods mentioned below.

1. Medications
Doctors are usually able to counter PAH by advising certain medications to their patients. The most effective medications for the disease include

• Prostanoids
  PAH causes the narrowing of arteries. And prostanoids help by widening this gap, which helps relax and open the pulmonary arteries. The medication does so by targeting a particular mechanism in the body, called the prostacyclin pathway. Prostanoids increase the amount of prostacyclin in the body, reducing the stress in the arteries.

• Endothelin Receptor Antagonists
  The narrowing of the pulmonary arteries in the lungs is caused due to a substance called endothelin. The endothelin receptor antagonists reverse the effects of endothelin and subsequently widen the arteries.

• Anticoagulants
  Medications that fall under this category prevent blood clots from forming. They interfere with the body’s blood coagulation mechanism, which ensures blood clots aren’t formed that easily.

• Soluble Guanylate Cyclase Stimulator
  A soluble guanylate cyclase stimulator promotes the interaction of soluble guanylate cyclase (sGC) and nitric oxide. sGC is a chemical in the body that interacts with nitric oxide to increase the blood flow in the lungs.

2. Oxygen Therapy
Oxygen may be administered using a mask or a pronged tube. It is placed just under the nose and delivers oxygen directly into it. While oxygen therapy may be administered only during sleep or during exercise, it can be administered more frequently than that as well. Your doctor might also suggest that you sometimes breathe pure oxygen to help treat pulmonary hypertension, especially if you live at a high altitude or have sleep apnea.

3. Surgery
Multiple surgical procedures can also help combat PAH. Here are two common procedures advised by doctors:

• Atrial Septostomy
  When even medications do not help control PAH, doctors recommend going ahead with this open-heart surgery. The goal of atrial septostomy is to relieve pressure on the right side of the heart. The surgeon does so by creating an opening between the upper left and right chambers of the heart (atria).

• Transplant
  If atrial septostomy doesn’t work, a lung or heart-lung transplant might be the only option. While the procedure is effective, it comes with major risks such as rejection of the transplanted organ.