A rare lung disorder, primary pulmonary hypertension (PPH) is a case where the blood vessels in the lungs become narrow, thus causing high blood pressure in the lungs. Pulmonary arteries are responsible for carrying blood from the body to the lungs. It is in the lungs where carbon dioxide is traded for oxygen.

Also known as idiopathic pulmonary arterial hypertension, PHN is a chronic disease and can lead to heart failure, if not treated on time.

Causes of primary pulmonary hypertension

Although causes underlying primary pulmonary hypertension are unknown, there are some forms of PHN that can be linked to defective genes that run in the family. Researchers have established that gene mutation often leads to sensitization of blood vessels and theses can narrow, or constrict when exposed to such factors.

The symptoms of PPH are as follows.

• Fatigue (extreme tiredness) and dizziness
• Loss of breath, trouble in breathing, especially during any physical activity.
• Persistent dry cough
• Swelling of the ankles or legs
• Bluish lips
• Angina (chest pain)
• High pulse
• Palpitations
• Hypoxia- the level of oxygen depletes in blood

The above-mentioned symptoms usually get worse as the disease progresses.

Less common symptoms of PPH

• Non-productive cough
• Exercise-induced nausea
• Vomiting
• Coughing up of blood

The signs and symptoms of primary pulmonary hypertension are unrecognizable in its early stages, and often go unnoticed for months. Signs of primary pulmonary hypertension (PPH) is often confused with other conditions that have an effect on the heart and lungs.

While diagnosing pulmonary hypertension, doctors ask about the symptoms and risk factors, including family history and other medical conditions. Having a family member who has or had pulmonary hypertension increases your chances of developing the disease.

PPH is usually diagnosed when a possibility of other diseases have been disregarded. Tests may include:

• Chest X-ray
  Pictures of internal tissues is taken to assess the current condition of the heart.
• Electrocardiogram (ECG)
  The aim of this test is to record the strength and scheduling of electrical activity of the heart. In this test, small sensors are taped to the skin which picks up the electrical activity, which is further used to detect damage to heart muscle.
• Echocardiogram (echo) 
  Sound waves are used to check the heart’s chambers and valves. With the help of sound waves, an ultrasound transducer is passed over the skin and heart; a picture is presented on a screen. Echo helps in explaining the level of enlargement and damage in the heart.
• Pulmonary function tests (PFTs)
  In order to measure lung’s ability to move air to and fro from the lungs, such diagnostic tests are performed. These tests are carried out with the help of special machines in which the patient breathes into, and the test assesses the current capacity.
• Perfusion lung scan
  Nuclear radiology test, this scan uses a small amount of a radioactive substance to find fluctuations in the arteries that are responsible for managing blood flow to the lungs. Perfusion lung scan also helps in evaluating the functioning of the lungs.
• Cardiac catheterization
  Normally done on the right side of the heart, in this procedure, a dye (contrast agent) is injected into the pulmonary artery and then an x-ray is taken. This method helps in identifying blockages or narrowing of arteries. This test is also used to measure the pressure inside respiratory arteries.
• Blood tests
  It helps in checking oxygen level in the blood. It is also used to evaluate the functioning of liver and kidney and check for other diseases. Certain blood tests help assess strain on the heart.

Treatment for primary pulmonary hypertension
Primary pulmonary hypertension (PPH) is non-curable. However, there are various treatment options available that help in managing the symptoms. Some of these are:

• Anticoagulants
  Blood thinners, anticoagulants reduce the density of the blood in order to reduce clotting and to allow it flow more freely.
• Diuretics
  These are water pills that help in decreasing the water content in the body. It reduces the load on the heart by reducing the swelling and by washing out extra fluids from the body.
• Vasodilator/calcium channel blocking drugs
  In order to improve heart’s ability to pump blood, doctors often prescribe these medications.
• Other drugs
  Certain medications are helpful in lowering blood pressure in the lungs. These medications help the heart pump more blood by making the heart beat stronger.
• Oxygen therapy
  In a case of severe PPH, breathing becomes difficult and in order to cope up with the same, supplemental oxygen is delivered through nasal prongs or mask.
• Lung or heart-lung transplant
  This is the ultimate option for people suffering from severe PPH. Combined heart and lung transplantation is limited to patients who are at the end stage and in whom it offers the only surgical option.