What is Pulmonary Hypertension and PAH?

"Pulmonary Hypertension" aka "PAH" (medical name is "Pulmonary Arterial Hypertension") is a very serious disease, which condition may be treated but not completely cured.

A good date to research Pulmonary Hypertension disease and learn about ways to Treat or Stop PAH from getting worse by starting today

pulmonary-hypertension pictures normal vs abnormal

Pulmonary arterial hypertension is excessive high blood pressure in the lung arteries. The condition forces the heart right side to work harder than it normally should.

Causes of Pulmonary Hypertension Disease

The right side of the heart pumps blood through the lungs, where it gets oxygen. Blood returns to the left side of the heart, where it is pumped to the "rest of the" body.

When the small arteries a.k.a. blood vessels of the lungs become narrowed, they cannot carry as much blood. When this happens, pressure builds-up. This is called pulmonary hypertension, or PAH.

The heart needs to work harder to force the blood thru the vessels against this extra pressure. Over time, this causes the right side of the heart to become larger. This condition is called right-sided heart failure, or cor pulmonale.

Pulmonary hypertension issues and possible causes:

  • Autoimmune diseases which may damage lungs, such as severe rheumatoid arthritis
  • Birth defects of the heart
  • Strokes and high stroke risk, so you should lower stroke risk to help avoid lung blood clots (pulmonary embolism) and clots elsewhere from causing a blockage
  • Heart valve disease
  • Heart failure
  • HIV infection
  • Lung disease, including COPD, or pulmonary fibrosis
  • Low oxygen levels in the blood for a long time (chronic)
  • Medicines (for example, some diet drugs)
  • Sleep apnea

If pulmonary hypertension is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension. In many PAH patients, the cause of pulmonary hypertension is not known. In this case, the condition is called idiopathic pulmonary arterial hypertension; i.e. idiopathic fibrosis. Idiopathic means the exact disease cause is not known. Idiopathic pulmonary hypertension is rare and affects more women than men for unknown reasons.

Symptoms of Pulmonary Hypertension

Shortness of breath or light-headedness during activity is often the first symptom. Fast heart rate (palpitations) may be present. As as the PAH condition worsens, symptoms may occur with light activity or even while resting.

Other symptoms include:

  • Chest pain or pressure, often in front of chest
  • Dizziness or fainting spells
  • Fatigue
  • Ankle and leg swelling
  • Bluish color of the lips or skin (cyanosis)
  • General weakness
  • Increased abdomen size

People with pulmonary hypertension often have come-and-go symptoms reporting both 'good days' and 'bad days'

Exams and Tests for Pulmonary Hypertension

A physical examination may show:

  • Abnormal heart sounds
  • Feeling of a pulse near the breastbone
  • General pain & swelling
  • Heart murmur on right-side of heart
  • Larger than typical veins in neck
  • Leg pain & stiffness
  • Liver and spleen swelling
  • Normal breathing sounds

In the early stages of PAH disease, the exam may be normal or almost normal. The condition may take several months to diagnose. Asthma and other diseases may cause similar symptoms and must be ruled out.

atherosclerotic cardiovascular disease of the heart

Medical testing may include:

  • 6-minute walk test
  • Blood tests
  • Cardiac catheterization
  • Chest x-ray
  • CT scan of the chest
  • Echo cardiogram
  • EKG & ECG
  • Lung failure and function testing
  • Nuclear medicine lung scan
  • Pulmonary arteriogram
  • Sleep apnea study
  • Treadmill heart and cardio stress test

Treatment for Pulmonary Hypertension

There is no cure for pulmonary hypertension. The goal of treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve problems.

Many treatment options for pulmonary arterial hypertension are available. If you are prescribed meds, they may be taken by orally, received through the vein (intravenous, or IV), or inhaled.

Your doctor will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you are responding to "the medicine." Do not stop taking your medicines without talking to your doctor.

Other treatments may include:

  • Blood thinners to reduce the risk of blood clots, especially if you have IPAH
  • Oxygen therapy at home
  • Heart-lung transplant, if medicines do not work

Other important tips to follow:

  • Avoid pregnancy
  • Stop smoking & avoid getting emphysema
  • Avoid heavy physical activities and lifting
  • Avoid traveling to high altitudes
  • Get a yearly flu vaccine, as well as other vaccines such as the pneumonia vaccine

Outlook/Prognosis for Pulmonary Arterial Hypertension

How well you do depends on what caused the condition.

As the illness gets worse, you will need to make changes in your home to help you get around the house.

When to Contact a Medical Professional

Call your health care provider if:

  • You begin to develop shortness of breath when you are active
  • Shortness of breath gets worse
  • You develop chest pain
  • You develop other symptoms

Alternative Names

Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary hypertension; Arterial Hypertension; Primary pulmonary hypertension; PPH; PAH; Secondary PAH.

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