Pulmonary hypertension

Pulmonary Hypertension


Hypertension in the pulmonary circuit (type of high blood pressure that affects only the arteries in the lungs and the right side of your heart).

Abbreviation: PH.

ICD-9: 416.0.

Alternative Names: Sporadic primary pulmonary hypertension, pulmonary arterial hypertension, familial primary pulmonary hypertension, and idiopathic pulmonary arterial hypertension.

Your heart has two upper and two lower chambers. Each time blood passes through your heart, the lower right chamber (right ventricle) pumps blood to your lungs through a large blood vessel (pulmonary artery). In your lungs, the blood releases carbon dioxide and picks up oxygen. The oxygen-rich blood then flows through blood vessels in your lungs (pulmonary arteries, capillaries and veins) to the left side of your heart.

Ordinarily, the blood flows easily through the vessels in your lungs, so blood pressure is usually much lower in your lungs. With pulmonary hypertension, the rise in blood pressure is caused by changes in the cells that line your pulmonary arteries. These changes cause extra tissue to form, eventually narrowing or completely blocking the blood vessels, making the arteries stiff and narrow. This makes it harder for blood to flow, raising the blood pressure in the pulmonary arteries.

PH is divided into five groups based on its causes. In all groups, the average pressure in the pulmonary artery is higher than 25 mmHg at rest or 30 mmHg during physical activity. The pressure in a normal pulmonary artery is about 15 mmHg at rest.


Group 1:

    Group 1 pulmonary arterial hypertension (PAH) that occurs without a known cause is often called primary PAH or idiopathic PAH (IPAH).

  • Group 1 PAH that occurs with a known underlying disease or condition is often called associated PAH. For example, PAH that occurs in a patient who has scleroderma might be called "PAH occurring in association with scleroderma," or simply "scleroderma-associated PAH."

Groups 2 to 5:

  • These are called secondary PH.


Symptoms Include, chest pain, fainting, dizziness, lightheadedness, shortness of breath, and weakness.

Causes and Risk Factors:

Pulmonary hypertension may be caused by:

  • Lung or heart valve disease.

  • Obstructive sleep apnea.

  • History of a blood clot in the lung.

  • Sickle cell anemia.

  • Cirrhosis.

  • Certain diet medications.

  • Left-sided heart failure.

  • Living at altitudes higher than 8,000 feet (2,438 meters).

  • Climbing or hiking to altitudes higher than 8,000 feet (2,438 meters) without acclimating first.

  • Use of certain stimulant drugs, such as cocaine.

Other conditions that may cause pulmonary hypertension include:


  • Systemic lupus erythematosus.

  • Sarcoidosis.

  • Human immunodeficiency virus (HIV).

  • Advanced liver disease.

Although anyone can develop pulmonary hypertension, older adults are more likely to have secondary pulmonary hypertension, and young people are more likely to have idiopathic pulmonary hypertension.

Another risk factor for pulmonary hypertension is a family history of the disease. Some genes could be linked to idiopathic pulmonary hypertension. These genes might cause an overgrowth of cells in the small arteries of your lungs, making them narrower.

If one of your family members develops idiopathic pulmonary hypertension and tests positive for a gene mutation that can cause pulmonary hypertension.


Pulmonary hypertension is hard to diagnose early because it's not often detected in a routine physical exam. Even when the disease is more advanced, its signs and symptoms are similar to those of other heart and lung conditions.

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.

Your doctor may do one or more tests to rule out other possible reasons for your condition. The first tests you'll have to diagnose pulmonary hypertension include:


  • Transesophageal echocardiogram (TEE).

  • Chest X-ray.

  • Right heart catheterization.

  • Pulmonary function test.

  • Overnight oximetry.

  • Perfusion lung scan.

  • Computerized tomography (CT) scan.

  • Magnetic resonance imaging (MRI).

  • Open-lung biopsy.


The treatment for pulmonary hypertension depends on the underlying cause.

  • If the COPD is the cause, then providing oxygen and appropriately treating the underlying lung disease by a pulmonologist is the first step in treatment.

    If left sided heart failure is the primary problem, then adequate treatment of the left heart failure by a cardiologist is the main stray of treatment.

    If scleroderma, which often can cause pulmonary hypertension, a rheumatologist is involved in the treatment program.

Some of the most commonly used drugs prescribed to treat pulmonary hypertension include:

1. Epoprostenol.

2. Bosentan.

3. Intravenous treprostinil.

4. Inhaled iloprost (Ventavis),

5. Calcium channel blockers (such as nifedipine).

6. Sitaxsentan, not FDA approved in the U.S.

7. Ambrisentan (Letairis).

Note: The following drugs and medications are in some way related to, or used in the treatment. This service should be used as a supplement to, and NOT a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners.

Surgeries Include:

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.

3. 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.

Making lifestyle changes can help you manage your symptoms.

1. Quit Smoking

2. Follow a Healthy Diet.

3. Do Physical Activity, such as walking.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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