Primary Pulmonary Hypertension or PPH is a rare condition which causes elevated blood pressure in your lungs. The pulmonary arteries carry deoxygenated blood from different parts of your body to your lungs. In PPH, the blood vessels in your lungs become narrow increasing the blood pressure in your artery much above the average levels. It is important that you seek medical attention immediately as this is a fatal condition and can even cause heart failure.

Causes Of Primary Pulmonary Hypertension

The primary cause of PPH is still unknown. But doctors believe that genetics play a significant role. A gene defect in the family can cause primary pulmonary hypertension. According to researchers, a mutation in the genes causes the blood vessels to become overly sensitive to some factors. And when the blood vessels are exposed to those factors, they begin to constrict.

Symptoms Of PPH
Most of the times, the symptoms of PPH take several years to develop, and each one experiences these symptoms differently. Have a look at some of the common symptoms of primary pulmonary hypertension.

• Dry cough
• Fainting and dizziness
• Bluish skin and lips
• A racing pulse
• Trouble in breathing, especially after some activity
• Pain in the chest
• Swelling of legs or ankles
• Fatigue or extreme tiredness
• Palpitation.

  The symptoms are likely to get worse with the progression of the disease. You can experience severe symptoms in the advanced stage of this disease, so much that you may suffer from the symptoms even when at rest or become bedridden.

In most cases, the symptoms of PPH look like any other medical problem. This makes PPH difficult to diagnose, and it is recommended that you get yourself examined by a reputed healthcare provider.

Diagnosis Of PPH
It is a rare condition and is difficult to detect in a regular medical routine. Also, as the disease advances, the symptoms can be mistaken for other conditions that affect the lungs and the heart. Primary pulmonary hypertension can be diagnosed only after ensuring that the symptoms don’t resemble any other disease.

Tests can include the following,

• Blood test
  This will help to check the oxygen levels in the blood, evaluate kidney and liver function, and look for other diseases. Also, certain blood tests can help assess the strain on the heart.
• Chest X-ray
  This takes pictures of the internal tissues, including the heart to understand the severity of your condition.
  Pulmonary function or PFTs
  These diagnostic tests determine the ability of your lungs to handle the movement of air as you breathe in and out. You will have to breathe into a special machine when you undergo this test.
• Perfusion lung test
  This nuclear radiology examination uses a radioactive substance in small quantities. This test will find any changes in the shape of the arteries of the lungs or the amount of blood flow in them. This test can also help understand the function of your lungs.
• Echocardiogram (echo)
  This test uses sound waves to check the heart’s chambers and valves. An ultrasound transducer is passed over the skin right above the heart, and the echo sound waves create an image on a screen. The echo can show any damage or enlargement of the heart.
• Cardiac catheterization of the heart
  In this procedure, a dye (contrast agent) is injected into your pulmonary arteries, and then X-Rays are taken to identify any blockages, narrowing or other changes. This is the only test that can measure the pressure inside your pulmonary arteries directly.
• Electrocardiogram (ECG)
  This test helps to record the strength and timing of the electrical activity of the heart. The doctor places small sensors on your skin to detect the electrical activity. This test can show abnormal heart rhythms and can sometimes even detect damage to the heart muscle.

Key Points Of Primary Pulmonary Hypertension
It is a rare lung disorder that increases the blood pressure in your lungs. The cause of primary pulmonary hypertension is still unknown but is highly dependent on genetics and mutations.
Its symptoms take several years to develop and can often go unnoticed. The symptoms become severe as the disease progresses. The treatments of PPH are only aimed to manage the symptoms. There is no substantial cure for this lung disorder.

In some cases, you may need an oxygen supplement for your lungs. This is done using nasal prongs. In severe cases, a lung transplant (sometimes including a heart transplant) is needed for proper functioning of the body. In any case, if you experience any of the symptoms of PPH, make sure that you seek medical attention at the earliest.