How do people get PAH?

Idiopathic PAH occurs without any apparent cause

Genetically Inherited means a family history of PAH.

Associated with PAH (Secondary PAH) is associated with other diseases. These can include:

• Collagen vascular disease (scleroderma and lupus primarily)
• Congenital heart disease
• Chronic liver disease
• Pulmonary Embolism – Blood Clot in the lungs

Less common causes

• Human immunodeficiency virus (HIV)
• Sickle cell disease
• Use of diet drugs such as fenfluramine or dexfenfluramine
• Use of cocaine, methamphetamine, or other street drugs
• Persistent pulmonary hypertension of the newborn
• Other (hemorrhagic hereditary telangectasias, glycogen storage disease, etc.)
• Associated with significant venous or capillary involvement: pulmonary venooclussive disease, pulmonary capillary hemangiomatosis, respectively.

How do you know if you have PAH?

PH is often not diagnosed quickly because its early symptoms can be confused with those of many other conditions, e.g. asthma, angina. Some of the symptoms of PH include:

• Progressive breathlessness or shortness of breath, especially with activity but also at rest.
• Blue lips, skin or fingernails
• Feeling tired all the time, Fatigue
• Light headedness, especially when climbing stairs or upon standing up
• Swollen ankles, legs, or abdomen
• Chest pain, especially during physical activity
• Fainting

If you have any of the above symptoms you should contact your GP, who will be able to refer you to a specialist and request testing.  However, anything that you consider to be an emergency call 111.

What tests will I need to have?

To establish a diagnosis of PH, a series of tests are performed that show how well a person’s heart and lungs are working. These tests may include:

• Chest x ray Assess heart size. Look at the lungs. Check for any underlying conditions which may be causing breathlessness, e.g. asthma, chest infection.
• Electrocardiogram (ECG, a recording of the electrical current in the heart)Electrodes placed on skin at specific points which pick up your heartbeat.Measures the electrical activity of the heart. May detect if the right ventricle muscle is growing in order to increase the amount of blood pumped to the lungs.
• Echocardiogram (ultrasound of the heart) A simple, safe, non invasive procedure.  Involves a small probe on the chest (similar to a pregnancy ultrasound).  Gives information about the anatomy (structure) and function of the heart.
• CT Scan You will have an injection of an x ray dye which gives a warm feeling through the body.  Scan takes about 5 minutes.  It looks at lung volume, lung tissue and blood vessels.
• Lung Function Test Involves blowing into a tube.  Measures how well your lungs are working.
• Right heart catheterization

  This is the direct measurement of pressure in the pulmonary arteries and determination of how well the heart is pumping blood to the body. It is the most invasive test. You will go into a room that looks a little like being in theatre. A local anaesthetic will be given.  A catheter tube is fed up into your heart and pulmonary artery through the femoral vein (in your groin) to measure the pressure.

• Six-minute walk test to assess exercise capacity Most common test.  Walking between two cones for six minutes.  Your distance, heart rate and oxygen levels with be measured and compared against the previous walk.

What treatment can I have?

• Sidenifil (Viagra) An oral tablet taken three times a day.
• Bosentan An oral tablet taken twice a day.
• Iloprost (ventavis) A liquid that is nebulised every three hours (6-7 times a day)
• Oxygen Often people start using oxygen at night only, and may progress to using it more frequently.
• Warfarin Oral tablets taken daily to thin the blood, need to have frequent blood tests to monitor the INR levels.  This is done through your G.P.
• Diuretics Usually oral tablets taken daily to prevent fluid retention.
• Atrial Septostomy A hole is made between the right and left atriums in the heart the procedure is similar to having a right heart catheter done.  It is not done frequently and can be high risk.
• Lung Transplant Reserved for patients who have failed medical treatment. Major Operation. Not suitable for some types of PAH e.g. Scleroderma.
• Pulmonary Thrombo- Endarterectomy Removal of blood clots from the lungs.
  

  
  For more information see:

  www.phassociation.org

  www.phassociation.uk.com

  www.phaaustralia.com.au