What is Pulmonary Hypertension?

Pulmonary hypertension is a condition characterized by increased pressure in the pulmonary artery.

Pulmonary arteries are large blood vessels that carry deoxygenated blood from the heart to the lungs. There, the blood is oxygenated and returns to the heart where it is pumped to the rest of the body.

Because the distance the blood travels between the heart and the lungs is short, the pressure in these vessels is typically low.

When the pressure in the pulmonary arteries gets too high, the blood flow decreases, which results in lower oxygen levels.

Someone who has pulmonary hypertension may have narrowed arteries in their lungs. This is a progressive condition, so people suffering from it will receive less oxygen over time.

This rare condition affects between 50 and 70 million people worldwide — about 1% of the world’s population.

What are the Risk Factors for Pulmonary Hypertension?

Because the symptoms of pulmonary hypertension may be mistaken for symptoms of other conditions, the presence of one or more of these risk factors may be grounds for further testing.

The risk of developing pulmonary hypertension increases due to:

• Genetics: A family history of heart disease, lung disease, blood clots, or congenital defects (these defects are present at birth).
• Habits: Smoking, recreational drug use, and alcohol abuse.
• Medication: The use of certain prescribed medications — for example, cancer drugs and antidepressants.
• Age: The older you are, the greater your chances of developing pulmonary hypertension become. It is typically diagnosed in patients between the ages of 30 and 60.
• Sex: Women are more likely than men to develop pulmonary hypertension if they’ve already been diagnosed with heart failure.
• Pre-existing conditions: This includes diagnoses that involve the heart or lungs, such as heart failure, congenital heart disease, coronary artery disease, interstitial lung disease, cystic fibrosis, and other conditions.
• Environment: Asbestos exposure and certain parasitic infections.

What are the Symptoms of Pulmonary Hypertension?

During the early stages of the disease, many symptoms of pulmonary hypertension overlap with other medical conditions, which makes it difficult to diagnose.

Research has shown that one-third of pulmonary hypertension patients receive a misdiagnosis, while more than two-thirds are diagnosed with a more severe disease, which may indicate a delayed diagnosis, late referral, or a previous misdiagnosis.

If you have any of the risk factors mentioned above, express your concerns to your doctor so that further testing can be done to rule out pulmonary hypertension.

Symptoms of pulmonary hypertension include:

• Shortness of breath, especially during activities that don’t typically tire you (this is the main symptom)
• Feeling faint, dizzy, or lightheaded
• Chest pain that feels like pressure, heaviness, or a squeezing sensation (angina)
• Increased heart rate or a pounding heart (heart palpitations)
• Swelling in the legs and ankles (edema)
• A bluish tint to the lips, skin, and nails (cyanosis)

What are the Five Different Types of Pulmonary Hypertension?

Pulmonary hypertension can be separated into five groups. These groups are classified based on the cause and mechanism of the condition.

Group 1: Pulmonary arterial hypertension (PAH)

Pulmonary arterial hypertension (PAH) is caused by any impairment or damage to the pulmonary artery itself or the smaller blood vessels branching off into the lungs.

Damage may include narrowing, blockages, or destruction of these vessels.

If the lungs are not supplied with enough blood, the lower right chamber of the heart (right ventricle) will begin to pump harder in order to correct this. This results in higher pressure in the pulmonary artery and the smaller vessels.

Only around 15 to 50 people per million have PAH in the U.S. and Europe.

It is also possible to develop pulmonary hypertension without having any previous health issues. This is called idiopathic pulmonary arterial hypertension (IPAH).

Group 2: Pulmonary hypertension caused by left-sided heart disease

This group is characterized by the inability of the left lower chamber to effectively pump blood out of the heart and to the rest of the body. This is due to any impairment of the left ventricle.

Impairments can include:

• Valvular heart disease: Dysfunction of the heart valves
• Left-sided heart failure: When the heart muscle is too weak to adequately pump blood to the organs that need it.
• Coronary artery disease: Damage or disease of the coronary artery
• Abnormal heart rhythms: Read our article on heart arrhythmia to learn more

Pulmonary hypertension is also a likely complication of congenital heart disease, which refers to problems with the heart structure that exist from birth.

Group 3: Pulmonary hypertension caused by lung disease and/or hypoxia

This group is characterized by an increase in pulmonary pressure due to dysfunction in the lungs. The smaller vessels that go into the lungs become narrow and constricted.

This leads to a lack of oxygen entering the body.

As a result, the heart tries to compensate by pumping harder. This raises the pressure in the pulmonary artery.

Conditions or impairments that may cause Group 3 pulmonary hypertension include:

• Chronic obstructive pulmonary disorder (COPD): A condition that causes the blockage of airflow out of the lungs.
• Pulmonary fibrosis: A lung disease that causes scarring and lack of elasticity in the lung tissue, which results in breathing difficulties.
• Cystic fibrosis (CF): A genetic condition where there is an excessive buildup of thick, sticky mucus in the lungs.
• Sarcoidosis: This rare inflammatory disease affects 150,000 to 200,000 people in the U.S. each year. It causes inflammation of various tissue groups in the body due to an immune system overreaction.
• Removal of a lung: Due to surgical intervention or trauma.

Group 4: Pulmonary hypertension caused by arterial obstructions

This group is characterized by blood clots. People with deep vein thrombosis (DVT) are commonly affected.

DVT occurs when a blood clot gets stuck in one of the veins in the lower leg. Once the clot moves or dislodges, it may end up in the pulmonary artery or other smaller blood vessels in the lungs (pulmonary embolism).

A blood clot obstructs blood flow in the vessel, which results in increased pressure in the pulmonary artery.

Group 5: Pulmonary hypertension caused by other health conditions

This happens when pulmonary hypertension is not the initial diagnosis but secondary to another heart or lung condition.

Examples of such conditions or impairments include, but are not limited to:

• Anemia: Lack of iron that causes decreased oxygen in the blood.
• Valvular heart disease: Impairment of the valves of the heart.
• Left ventricular dysfunction: Impairment of the lower left heart chamber.
• Hypoxic vasoconstriction: A closing off of the smaller vessels in parts of the lung that are no longer functioning, and redirecting the blood to healthier parts of the lung.
• Interstitial lung disease: Scarring and lack of elasticity in lungs.

How Severe is Pulmonary Hypertension?

The severity of pulmonary hypertension is determined based on the average pressure in the pulmonary artery. A pulmonary pressure reading of 35 mm Hg or higher is considered pulmonary hypertension.

A cardiologist will perform a right heart catheterization to make a diagnosis.

Can Pulmonary Hypertension Affect Newborns?

While in the womb, blood usually bypasses the lungs as the baby gets oxygen from their mother.

Once the child is born, their circulatory system must adapt to getting air from their own lungs.

If this process does not happen effectively, pulmonary artery pressure increases, which leads to persistent pulmonary hypertension in newborns (PPHN). The condition affects one in every 1,250 babies.

The mortality rate in these cases ranges between 5-10%, even with appropriate intervention. Neurodevelopmental delays in infants with PPHN have also been reported.

Treatment and management for newborns with PPHN

After the acute (immediate) medical management and stabilization, the baby should be assessed for any health problems as they grow older. Should your child display signs of cognitive and neurodevelopmental delay due to persistent pulmonary hypertension, a consultation with an occupational therapist is typically recommended.

What are the Treatments for Pulmonary Hypertension?

There is currently no cure for pulmonary hypertension, but there are several things you can do to manage the condition and improve your quality of life.

Medication

Treatments prescribed by your doctor may include the following:

• Anticoagulants — such as warfarin and heparin — which decrease the rate at which the body forms blood clots. These are especially helpful in pulmonary hypertension caused by pulmonary embolisms.

• Sildenafil (Viagra) and other phosphodiesterase inhibitors are also useful as they directly dilate the pulmonary artery, which makes it easier for blood to flow.

• Diuretics like bumetanide (Bumex), furosemide (Lasik), and torsemide (Demadex). These drugs assist the kidneys in removing excess fluid and salt from the blood. It is commonly referred to as “water pills''.

• Calcium channel blockers like amlodipine (Norvasc), verapamil (Veralan), and diltiazem (Cardizem). These drugs reduce the amount of calcium that can be absorbed by the body.

When calcium is absorbed by the heart and the surrounding vessels, it increases the vessels’ ability to contract — and when the blood vessels contract, they narrow and blood pressure goes up.

Calcium blockers disrupt the movement of calcium through calcium channels, which allows the blood vessels to relax and contract at a more appropriate rate, which in turn results in a decrease in pulmonary hypertension.

• Pulmonary vasodilators like nitric oxide (INoMax or Genosyl), iloprost (Ventavis), and treprostinil sodium (Remodulin). These medications are currently the only inhaled vasodilators that have been approved by the Food and Drug Administration (FDA).

These medications help the blood vessels relax, thereby increasing their diameter and allowing for more efficient blood flow. This lowers the resistance in the blood vessels and results in lower pulmonary hypertension.

Oxygen therapy

Oxygen helps keep blood vessels open by lowering vascular resistance, which then results in lower pressure in the pulmonary artery.

Oxygen therapy would be particularly helpful to pulmonary hypertension patients who live at higher altitudes where the level of oxygen in the air is lower.

Dietary and lifestyle changes

Managing chronic conditions, staying active, and maintaining a balanced diet can help manage several health conditions, including pulmonary hypertension.

Surgical interventions

If medication and lifestyle changes are ineffective, surgery is also an option. One of the following procedures may be performed:

• Pulmonary endarterectomy: Surgically removing blood clots formed in the lungs and vessels to reduce pulmonary arterial hypertension when it is caused by a pulmonary embolism.

• Atrial septostomy: Surgically making a hole between the two upper chambers of the heart. This assists by decreasing the pressure on the right side of the heart and lowering pulmonary hypertension.

Where Can I Learn More About Pulmonary Hypertension?

Don’t wait for the symptoms to worsen before a pulmonary hypertension diagnosis is ruled out. If you have one or more of the risk factors mentioned in our article, LifeMD can help.

Book your appointment today to speak with a licensed healthcare provider.