Pulmonary Hypertension

Pulmonary hypertension is a condition that happens when the small- and medium-sized blood vessels in the lungs become stiff and thick or blocked. This causes blood pressure in the lungs to rise, and forces the right side of the heart to work harder to get blood to the lungs.

Over time, the disease can cause serious damage to the lungs, heart and arteries. It also increases the risk of blood clots in the lungs.

When the walls of the blood vessels are too thick, oxygen and carbon dioxide cannot properly flow in and out of the blood stream. Less oxygen in the blood causes arteries to narrow even more, making the high blood pressure worse.

Over time, damage to the heart occurs. The muscles of the lower right chamber (the right ventricle) become thicker and larger (a condition called cor pulmonale). They are not able to pump blood efficiently. This can cause heart failure.

Blood pressure measurements taken on your arm are not directly related to blood pressure in your heart and lungs. Pulmonary blood pressure is focused on the blood flow between your heart and lungs.

Pulmonary hypertension is broken down into different types. They include:

  • Pulmonary arterial hypertension, or PAH. This includes cases with no known cause, pulmonary hypertension that was inherited, and pulmonary hypertension caused by medications or drugs.
  • Pulmonary hypertension due to heart disease. The condition may occur because of problems with the ventricles or valves of the heart.
  • Pulmonary hypertension due to lung disease. Chronic obstructive pulmonary disease, such as emphysema, can cause pulmonary hypertension. Diseases that cause damage and scarring of lung tissues and disordered breathing during sleep can also result in pulmonary hypertension.
  • Chronic thromboembolic pulmonary hypertension. This type of PH is caused by blood clots in another part of the body traveling to the lungs. Sometimes people treated for these clots do not fully recover, and the clots cause the blood pressure in the lungs to rise.

Symptoms

Early symptoms of pulmonary hypertension are often mistaken for being out of shape, such as:

  • Shortness of breath when active
  • Lightheadedness
  • Tiring easily
  • Chest pain like angina
  • Achy joints

Patients who have an underlying cause for the condition may have coughing and wheezing.

When the heart has been damaged by the condition, a person may have swelling in their legs.


Causes and Risk Factors

Pulmonary hypertension has no known cause in most cases. Women are twice as likely as men to develop pulmonary hypertension. Half of people with the disease are diagnosed after age 35.

Other lung conditions may cause pulmonary hypertension. Diseases that interfere with the flow of blood in the lungs or the amount of oxygen that gets into the blood may cause pulmonary hypertension. Diseases that are sometimes linked with pulmonary hypertension include chronic obstructive pulmonary disease, cystic fibrosis or occupational lung diseases.

Other causes can include:

Diagnosis

Diagnosis of pulmonary hypertension can be difficult. Patients often do not notice their symptoms until they have already had serious damage to their heart and lungs.

A doctor will do a physical exam and review medical history and symptoms. Your doctor will have a few goals while trying to diagnose the disease.

  • Confirm that pulmonary hypertension is causing the symptoms and figure out how severe the disease is.
  • Look for what caused the blood pressure in the lungs to rise.
  • Make a treatment plan based on the type of pulmonary hypertension, the cause, how far along the disease is, and the specific needs of the patient.

Doctors may also order tests to help confirm the diagnosis.

Chest X-rays are often used to confirm the diagnosis and better understand the patient’s condition by providing a picture of what is going on inside the lungs. These images may be able to show if the pulmonary arteries have become enlarged.

Doctors may also use an electrocardiogram or echocardiogram to check for heart problems, such as thickening of the right ventricle or abnormal blood flow.

Blood tests to check oxygen levels and lung function tests may also be used.

The best way to confirm that pulmonary hypertension is present is to measure the blood pressure in the pulmonary artery. This is done by passing a tube (a wire catheter) through a vein in the neck, arm or leg into the right side of the heart to measure the blood pressure in the right ventricle and in the pulmonary artery.

Treatments

Pulmonary hypertension can often be well managed in patients through a combination of therapies, lifestyle changes, and other medical interventions as needed.

Each person with pulmonary hypertension needs a care plan based on their needs and health condition. The experts in Cedars-Sinai’s Pulmonary Hypertension Program can help.

Early treatment can reduce the chances of heart and lung damage.

Three types of medications are commonly used to treat pulmonary hypertension. They include:

  • Blood vessel dilators, which help widen blood vessels.
  • Drugs that prevent blood clots by increasing the levels of a certain chemical messenger that can prevent blood clots from forming.
  • Endothelin receptor antagonists, which stop a substance in the walls of the blood vessels that causes them to tighten and narrow.

Other medications and treatments also may be used, especially if the pulmonary hypertension is caused by an underlying condition. Other medications may include:

  • Medications that relax the blood vessels in the lungs
  • Calcium channel blockers, which help relax the muscles in the blood vessel walls. Only a small number of people respond to these.
  • Anticoagulants, sometimes called blood thinners, to stop clots from forming.
  • Diuretics, also called water pills, which help the body get rid of extra fluid.
  • An oxygen mask or other device to help relieve shortness of breath and lower blood pressure in the lungs.

Some patients in late stages of the disease may receive a single or double lung transplant or a heart and lung transplant.

People with pulmonary hypertension may improve their quality of life by making changes in their daily routine, such as quitting smoking and remaining a physically active as possible without worsening symptoms.

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