PAH or pulmonary arterial hypertension is also known as primary pulmonary hypertension. It is a very rare hypertension disease that affects your capillaries and arteries. The blood from the right ventricles of your heart flows into your lungs through these vessels. When the pressure in your arteries arises, your heart has to work much harder to pump the blood and as a result, it weakens the heart muscles. This can result in death as well.

Symptoms
During the early stages of the primary pulmonary hypertension, you will not get any such noticeable symptoms. However, as your condition gets from bad to worse, there will be some symptoms that will get more and more noticeable. These symptoms include:

• Dizziness
• Chest pressure
• Fainting
• Fatigue
• Difficulty breathing
• Heart palpitations
• Rapid pulse
• Chest pain
• Bluish spots on your skin and lip
• Swelling of legs and ankles
• Swelling of your abdomen towards the later part of your disease

You will find it difficult to breathe when you exercise or perform any other type of physical activity. Also, you might find it difficult to breathe even at the time when you are taking rest.

Causes
PAH happens when the capillaries and the arteries which carry the blood from the heart to the lungs gets destroyed or constricted. This happens due to multiple reasons. But the exact reason for primary pulmonary hypertension is yet to be discovered by the doctors and scientists. According to research, almost 15% to 20% of the people who suffer from PAH in the country have it hereditarily. This is due to the mutation that takes place in the BMPR2 gene or other genes. This genetic mutation can continue to happen from generation to generation leaving someone a chance to develop this disease at the later part of their life. However, there are other medical conditions that can be associated as the potential cause for PAH. These medical conditions include:

• Disorders with certain connective tissue
• Some infections, such as schistosomiasis or HIV infection
• Chronic liver disease
• Congenital heart disease
• Certain drugs such as recreational drugs
• Fat burners
• Certain toxins in your body

These are not the only causes for PAH to develop. As already stated, there is no specific cause for PAH, or even if it’s there, it is still unknown to the doctors and scientists. When PAH develops without any related reasons, it is called idiopathic PAH.

Diagnosis
When your doctor thinks that you have primary pulmonary hypertension, he or she will ask you to do certain tests to assess the condition of your arteries and heart. These tests for PAH diagnosis will include:

• Chest X-ray: To know whether the pulmonary arteries have got enlarged or not.
• MRI or CT scan: To search for blood clots in your pulmonary arteries.
• Electrocardiogram: To identify any sign of abnormal rhythm or strain in your heart.
• Echocardiogram: To check the structure of your heart and how it is functioning and also measure the pressure of your pulmonary artery.

You doctors will make you go through these tests just to find out the signs of PAH and also other causes for the symptoms you are having. Doctors generally look to rule out any other probable causes before determining that you are suffering from PAH.

Treatment
At present, there is no permanent cure for primary pulmonary hypertension. However, there are certain treatment options which will decrease the risk of complications and get you a prolonged life.

• Medications: PAH is sometimes related to other medical conditions that you may have. In such a case, your doctor will prescribe you certain medications. Also, if you are currently taking any medication, then the doctor will try to adjust these PAH medications along with them.
• Surgery: Surgical treatment is another option for PAH treatment. But, this treatment option will depend on the severity of the condition you have. Arterial Septostomy could be done in order to reduce the pressure on the right side of the heart. Also, the replacement of the heart or lungs could be an option too.
• Changes in lifestyle: Another important aspect of PAH treatment is the lifestyle change without which no treatment option will be effective. The patients of PAH need to start living a more healthy lifestyle. This will include developing healthy food habits, exercising daily, maintaining the right weight according to the height, and finally quit the habit of tobacco smoking once and for all.

PAH is one such disease that worsens with time. Therefore, early detection and treatment will increase the life expectancy of the patient. That is why a medical consultation with your doctor is extremely important if you have any of the aforementioned conditions.