Pulmonary arterial hypertension (PAH) involves abnormally high blood pressure (hypertension) in the pulmonary artery.  The pulmonary artery carries blood from the heart (right ventricle) to the lungs.  When the smallest of these vessels become narrower in diameter (perhaps due to disease), this increases resistance to blood flow through these vessels.  This increases the pressure inside these vessels as well as the right ventricle.  Through time, the right ventricle has to pump harder and harder to move blood into the pulmonary artery, causing the heart to become enlarged and weakened.  Eventually, the heart (right ventricle) becomes so weakened that it can no longer pump blood and heart failure occurs.

Other names for PAH include Ayerza syndrome, Familial (heritable) primary pulmonary hypertension (FPPH), Idiopathic pulmonary hypertension (arterial), Primary pulmonary hypertension and Sporadic primary pulmonary hypertension.

Pulmonary Arterial Hypertension Causes

As there are many causes of Pulmonary Arterial Hypertension, we’ll keep the list general:

  • Any number of heart diseases can generate PAH
  • Viral infections such as HIV
  • Genetic mutations (this is known as familial or heritable PAH)
  • Drugs such as cocaine and amphetamines and some prescription diet medications
  • Scleroderma (autoimmune disease) which is a connective tissue disease that causes fibrosis
  • Chronic liver diseases
  • Unknown cause (can’t find a cause) which is referred to as idiopathic PAH

Pulmonary Arterial Hypertension Symptoms

Symptoms of PAH present when the increased blood pressure can’t fully overcome the increased resistance of the pulmonary arteries.  This not only has effects on the heart and lungs but to the entire body.

In early phase PAH, patients may experience shortness of breath known as dyspnea during exercise along with possible fainting.  As the disease progresses, these symptoms will occur even when patients are at rest:

  • Dizziness
  • Swelling of the legs and ankles known as edema.
  • Pain in the chest region.
  • tachycardia (fast heart rate) and irregular heart beat (palpitations or arrhythmias).

Pulmonary Arterial Hypertension Diagnosis

A doctor will ask potential PAH patients about family and medical history, and perform a physical exam and determine symptoms.  From there s/he will try to confirm PH with tests, such as an echocardiogram, chest X-ray, electrocardiogram (EKG) and/or right heart catheterization.  To try and determine the underlying cause, a doctor may perform Lung function tests, chest CT and MRI scans, and/or Polysomnograms.  Please refer to the introduction on PH for an explanation of these various procedures.

Pulmonary Arterial Hypertension Treatments

Currently, there is no cure for any of the pulmonary hypertension types, however there are treatments that can help mitigate with symptoms.  What therapies or combination of therapies are used depend on the underlying cause(s).  The goals of such treatments ares to:

  • Treat the underlying cause if it is known.  It is reported that some therapies can correct some forms of PAH in individuals with secondary pulmonary arterial hypertension (SPAH).
  • Improve the quality of life by reducing symptoms.
  • Reduce the heart’s workload by increasing the supply of blood and oxygen.
  • Reduce the production of blood clots and slow down the proliferation of smooth muscle cells in the blood vessel walls.

Treatments that are available include prescription drugs, oxygen therapy, lung transplants and possibly stem cell therapy.

Pulmonary Arterial Hypertension Medications

  • Blood thinners are commonly used to decrease the production of blood clots.
  • Calcium channel blockers help to relax blood vessels.  This allows for an increase in blood flow and oxygen to the heart.
  • Prostacyclin is administered to relax arteries in the lungs and prevents blood clots from forming.  There are several forms of this medication.  Newer forms of prostacyclin can be inhaled through a nebulizer for fast symptomatic relief.
  • Nitric oxide, which can be inhaled has the ability to relax pulmonary arteries.
  • Diuretics which help to remove excess fluid from the body reducing edema.
  • Phosphodiesterase 5 inhibitors (PDE5) allows blood vessels to relax in the lungs.

Oxygen Therapy

As PAH progresses, blood oxygen levels decline, so patients may need oxygen therapy.  The oxygen is administered through a mask or nasal prongs.

Lung Transplantation

If medical treatments are no longer an option for a PAH patient, they may require surgery to replace the diseased lung(s).  A donor is required.  Patients will have to take medication for the rest of their life to reduce the risk of rejection.

Pulmonary Arterial Hypertension Prognosis

Progress is being made to bring about new diagnostic tools and more efficient medications, however PAH is a fatal disease. It is reported that the survival rate is around 50 percent after 5 years.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.