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What Is It ?

High blood pressure in the arteries that supply the lungs is called pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH). The blood pressure measured by a cuff on your arm isn’t directly related to the pressure in your lungs. The blood vessels that supply the lungs constrict and their walls thicken, so they can’t carry as much blood. As in a kinked garden hose, pressure builds up and backs up.   The heart works harder, trying to force the blood through. If the pressure is high enough, eventually the heart can’t keep up, and less blood can circulate through the lungs to pick up oxygen. Patients then become tired, dizzy and short of breath.

  • The portion of the circulation that distributes oxygen-rich blood from the left side of the heart , throughout the body , is referred to as the systemic circulation .
  • The blood then returns from the body to the right side of the heart and passes through the lungs to replenish oxygen.
  • It then returns to the left side of the heart for another round through the systemic circulation.
  • The portion of the circulation that distributes the blood from the right side of the heart to the lungs is referred to as the pulmonary (lung) circulation.
  • The pulmonary arteries are the major blood vessels that carry blood from the right side of the heart to the lungs.

What Causes Pulmonary Hypertension?

Pulmonary hypertension can be caused by diseases of the heart and the lungs, such as:

  • chronic obstructive pulmonary disease (COPD)
  • emphysema,
  • failure of the left heart ventricle,
  • recurrent pulmonary embolism (blood clots traveling from the legs or pelvic veins obstructing the pulmonary arteries or chronic thromboembolic pulmonary hypertension), or
  • underlying diseases such as scleroderma.
Other conditions that may cause pulmonary hypertension include:

  • dermatomyositis,
  • systemic lupus erythematosus,
  • sarcoidosis,
  • human immunodeficiency virus (HIV), and
  • advanced liver disease (porto-pulmonary hypertension).

Pulmonary hypertension can also be caused by chronic low blood oxygen levels as in some patients with sleep apnea or other long-standing (chronic) lung disease.

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What Are The Signs And Symptoms Of Pulmonary Hypertension?

Signs and symptoms of pulmonary hypertension (PH) may include:

  • Shortness of breath during routine activity, such as climbing two flights of stairs
  • Tiredness
  • Chest pain
  • A racing heartbeat

As PH worsens, you may find it hard to do any physical activities. At this point, other signs and symptoms may include:

  • Feeling lightheaded, especially during physical activity
  • Fainting at times
  • Swelling in your legs and ankles
  • A bluish color on your lips and skin

How Is Pulmonary Hypertension Diagnosed?

Your doctor will diagnose pulmonary hypertension (PH) based on your medical and family histories, a physical exam, and the results from tests and procedures.

PH can develop slowly. In fact, you may have it for years and not know it. This is because the disease has no early symptoms.

When symptoms do develop, they're often like those of other heart and lung conditions, such as asthma. This makes PH hard to diagnose.

Medical And Family Histories

To learn about your medical history, your doctor may ask about your signs and symptoms and how and when they began. He or she also may ask whether you have other medical conditions that can cause PH.

Your doctor also may ask whether you have any family members who have or have had PH. People who have a family history of PH are at increased risk for the condition.

Physical Exam

During the physical exam, your doctor will listen to your heart and lungs with a stethoscope. He or she also will check your ankles and legs for swelling and your lips and skin for a bluish tint. These are signs of PH.

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Diagnostic Tests And Procedures

You may need tests and procedures to confirm a diagnosis of PH and to look for the underlying cause of the disease. Your doctor also will use test results to find out the severity of your PH.

Tests and Procedures To Confirm a Diagnosis :-


Chest x ray

EKG (electrocardiogram)

Right heart catheterization

Tests To Look for the Underlying Cause of Pulmonary Hypertension :-

Lung function tests

Overnight oximetry

Lung ventilation/perfusion (VQ) scan

Blood tests

Exercise testing

Finding Out The Severity Of Pulmonary Hypertension

How Is Pulmonary Hypertension Treated ?

Pulmonary hypertension (PH) has no cure, but treatment may help relieve symptoms and slow the progress of the disease.

PH is treated with medicines, procedures, and other therapies. Treatment will depend on what type of PH you have and how severe it is. (For more information, see "Types of Pulmonary Hypertension.")


If you have group 1 PAH, you may need medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. As the blood vessels relax, more blood can flow through them.

Examples of these medicines include phosphodiesterase-5 inhibitors, prostanoids, endothelin receptor antagonists, and calcium channel blockers.

To find out which of these medicines will work best, you'll likely have an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medicines. This test is done during right heart catheterization.

Medical And Surgical Procedures

If you have group 1 PAH, you may need one or more of the following procedures.

Atrial Septostomy : For this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria (the two upper chambers of your heart). This wall is called the septum.

A tiny balloon on the tip of the tube is inflated to create an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow. Atrial septostomy is rarely done in the United States.

Lung Transplant : A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.

Heart–Lung Transplant : A heart–lung transplant is surgery in which both the heart and lung are replaced with healthy organs from a deceased donor.

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