PULMONARY ARTERIAL HYPERTENSION (PAH)

"Pulmonary hypertension" describes high blood pressure in the arteries of the lungs. Pulmonary arterial hypertension (PAH) is one form of pulmonary hypertension. Pulmonary arteries carry blood from your heart to the lungs, where it picks up oxygen to be delivered throughout your body. In PAH, the pulmonary arteries constrict abnormally. This forces your heart to work faster and causes blood pressure within the lungs to rise.

There are several types of PAH. It can be caused by or occur at the same time as a variety of other medical problems. PAH also can be the result of taking certain medicines. The cause of some cases of PAH is unknown. PAH worsens over time and is life-threatening because the pressure in a patient's pulmonary arteries rises to dangerously high levels, putting a strain on the heart. There is no cure for PAH, but several medications are available to treat symptoms.

What are the Symptoms of Pulmonary Arterial Hypertension?

Symptoms may include:
  • Chest pain, usually in the front of the chest
  • Dizziness
  • Fainting
  • Fatigue
  • Leg swelling
  • Light-headedness during exercise
  • Shortness of breath during activity
  • Weakness
The symptoms of pulmonary arterial hypertension (PAH) are similar to those of other, more common, heart and lung problems. Those disorders include asthma, pneumonia, chronic obstructive pulmonary disease (COPD), left heart failure and coronary disease. For this reason, it may be difficult for a doctor to diagnose PAH. Patients and doctors might overlook the disorder until symptoms become severe.

How is pulmonary arterial hypertension diagnosed?

There is not one specific way to diagnose PAH. In early stages of the disease, a physical exam may be normal or almost normal. It may take your doctor several months to diagnose PAH. A doctor who suspects a patient may have PAH usually runs a series of tests to measure blood pressure in the pulmonary arteries, decide how well the heart and lungs are working, and rule out other diseases.

Tests include:
  • Chest X-Ray (of the heart and lungs)
  • Electrocardiogram (an electrical measure of your heart rate and how regularly your heart is beating)
  • Echocardiogram (an ultrasound picture of the heart to help determine how well it is working)
  • Right-heart catheterization (a special device that the doctor threads through a vein into the heart and arteries of the lungs to measure pressure in the arteries and test how well the heart is pumping)
  • Pulmonary function tests (breathing tests that measure lung function)
  • Perfusion lung scan (an image that shows blood flow through the lungs)
  • Six-minute walk test (a measure of the patient's ability to do exercise)

How is pulmonary arterial hypertension treated?

There is no cure for PAH, but there are several treatment options. Treatments include medications and surgery; patients also make lifestyle changes as their PAH worsens and limits daily activity. Treatments for PAH aim to reduce symptoms, improve quality of life, and slow down how quickly the disease progresses.

If your pulmonary hypertension is caused by another condition, then your doctor will also treat that underlying disease.

Medication

There are several types of drugs available to treat PAH. Each works differently, but all work to reduce the heart's workload. The effect of the medication is for blood to flow more easily through the pulmonary arteries. Your doctor may choose a type of medication based on how severe your PAH is and how well you respond to treatment. Sometimes medications are combined. Your doctor will decide which medicine is best for you. You doctor will monitor you closely to watch for side effects and how well you respond to the medication.


Types of medications your doctor may prescribe to ease symptoms include:

  • Endothelin receptor antagonists (ERAs) to stop the harmful effects of endothelin, a hormone that helps control blood flow and cell growth in blood vessels.  Patients with PAH often have high levels of endothelin.
  • Phosphodiesterase-5 inhibitors (PDEI) to relax the muscles and reduce abnormal cell growth in blood vessels
  • Prostacyclins to relax blood vessels, reducesabnormal cell growth, and help prevent blood clots
  • Anticoagulants to prevent blood clots in the lungs
  • Calcium channel blockers to reduce constriction in the pulmonary arteries
  • Diuretics to improve how effectively the heart works by reducing the volume of blood it must pump
  • Digoxin to help the heart pump more forcefully inhaled oxygen to reduce shortness of breath

Surgery

Your doctor may consider a surgical treatment for you based on many factors. Two surgical treatment  options are:

  • Lung or heart-lung transplantation--Patients who fail standard therapies may be candidates for heart-lung transplantation. Transplantation can be lifesaving, but the supply of donor organs is very limited.
  • Atrial septostomy—In this treatment, a surgeon creates a hole between the top chambers of the heart. This allows blood to be pumped to the rest of the body without going through the lungs first. This can reduce strain on the heart, but it also greatly reduces the amount of oxygen that can be delivered to the body.

Lifestyle Changes

As PAH symptoms worsen, people may need to make changes in their homes and daily activities. They also may need help around the home as they need to limit activity.


Further information can be obtained from:


PH Central  (www.phcentral.org)


Pulmonary Hypertension Association  (www.phassociation.org)

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