Pulmonary Hypertension

It is a serious condition. If you have it, the blood vessels that carry blood from your heart to your lungs become hard and narrow.​

Your heart has to work harder to pump the blood through. Over time, your heart weakens and cannot do its job and you can develop heart failure.

SYMPTOMS OF PH INCLUDE

• Shortness of breath during routine activity, such as climbing two flights of stairs
• Tiredness
• Chest pain
• A racing heartbeat
• Pain on the upper right side of the abdomen
• Decreased appetite

As PH worsens, you may find it hard to do any physical activities.

There are two main kinds of PH. One runs in families or appears for no known reason. The other kind is related to another condition, usually heart or lung disease.

There is no cure for PH. Treatments can control symptoms. They involve treating the heart or lung disease, medicines, oxygen, and sometimes lung transplantation.

Risk Factors

Group 1 pulmonary arterial hypertension (PAH) without a known cause is rare. It affects women more often than men. People who have group 1 PAH tend to be overweight.

PH that occurs with another disease or condition is more common.

PH usually develops between the ages of 20 and 60, but it can occur at any age. People who are at increased risk for PH include:

• Those who have a family history of the condition.
• Those who have certain diseases or conditions, such as heart and lung diseases, liver disease, HIV infection, or blood clots in the pulmonary arteries.
• Those who use street drugs (such as cocaine) or certain diet medicines.
• Those who live at high altitudes.

Causes

Pulmonary hypertension (PH) begins with inflammation and changes in the cells that line your pulmonary arteries. Other factors also can affect the pulmonary arteries and cause PH.

FOR EXAMPLE, THE CONDITION MAY DEVELOP IF:

• The walls of the arteries tighten.
• The walls of the arteries are stiff at birth or become stiff from an overgrowth of cells.
• Blood clots form in the arteries.

These changes make it hard for your heart to push blood through your pulmonary arteries and into your lungs. Thus, the pressure in the arteries rises, causing PH.

Many factors can contribute to the process that leads to the different types of PH.

Group 1 pulmonary arterial hypertension (PAH) may have no known cause, or the condition may be inherited.

Some diseases and conditions also can cause group 1 PAH. Examples include HIV infection, congenital heart disease, and sickle cell disease. Also, the use of street drugs (such as cocaine) and certain diet medicines can lead to PAH.

MANY DISEASES AND CONDITIONS CAN CAUSE GROUPS 2 THROUGH 5 PH (OFTEN CALLED SECONDARY PH), INCLUDING:

• Mitral valve disease
• Lung diseases, such as COPD (chronic obstructive pulmonary disease)
• Sleep apnea
• Sarcoidosis

Symptoms

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
• Pain on the upper right side of the abdomen
• Decreased appetite

As PH worsens, you may find it hard to do any physical activities.

AT THIS POINT, OTHER SIGNS AND SYMPTOMS MAY INCLUDE:

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

Diagnosis

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 condition has no early signs or symptoms.

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

MEDICAL AND FAMILY HISTORIES

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 will want to know whether you have any family members who have or have had PH. People who have a family history of PH are at higher 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 color. These are signs of PH.

DIAGNOSTIC TESTS AND PROCEDURES

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

Treatment

Pulmonary hypertension (PH) has no cure. However, 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 its severity. 

Several treatments may be used for all types of PH.

THESE TREATMENTS INCLUDE:

• Diuretics, also called water pills. These medicines help reduce fluid buildup in your body, including swelling in your ankles and feet.
• Blood-thinning medicines. These medicines help prevent blood clots from forming or getting larger.
• Digoxin. This medicine helps the heart beat stronger and pump more blood. Digoxin sometimes is used to control the heart rate if abnormal heart rhythms, such as atrial fibrillation or atrial flutter, occur.
• Oxygen therapy. This treatment raises the level of oxygen in your blood.
• Physical activity. Regular activity may help improve your ability to be active. Talk with your doctor about a physical activity plan that’s safe for you.

Recovery

Pulmonary hypertension (PH) has no cure. However, you can work with your doctor to manage your symptoms and slow the progress of the disease.