Pulmonary Hypertension

What is pulmonary hypertension?

Pulmonary Hypertension (HTN) occurs when the pressure in the blood vessels between the lungs and the heart is too high. PH causes the arteries in the lung to narrow and results in narrowed blood flow to the lungs and lower levels of oxygen in the blood. Because of this, blood flow to your heart is disrupted and as a result, your heart must work harder to pump oxygen-poor blood to your lungs. Overtime, PH can cause significant damage to your heart and body and can be fatal without treatment.

Pulmonary hypertension is divided into five groups based on its cause:

Group 1 PH due to pulmonary arterial hypertension (PAH) – makes your pulmonary arteries become narrow, thick or stiff. Less blood is able to flow through, which raises the pressure in your pulmonary arteries. Common causes range from underlying diseases to certain drugs.
Group 2 PH due to left-sided heart disease – the left side of your heart is responsible for pumping blood to your entire body. When there is a problem on this side of the heart, it affects the right side of your heart and your entire pulmonary circuit. This causes blood to back up in the heart, raising the pressure in your pulmonary arteries.
Group 3 PH due to lung disease or hypoxia – certain lung problems can cause the arteries in your lungs to tighten, resulting in less blood flow through your lungs and raising the pressure in your pulmonary arteries.
Group 4 PH due to blockages in your lungs – certain blockages, like blood clots or scars can prevent your blood from flowing normally through your lungs.
Group 5 PH due to other disorders – certain conditions, like blood disorders or metabolic disorders, can contribute to the development of PH.

What causes pulmonary hypertension?

Pulmonary hypertension can affect adults at any age. It is most common among people who already have heart or lung conditions. PH is dangerous for anyone, but especially for people who are pregnant. It can cause complications for both the parent and fetus. Other serious problems associated with PH include:

Anemia.
Arrhythmias.
Blood clots in your pulmonary arteries.
Pericardial diffusion.

Common causes can range from diseases and underlying conditions to environmental exposures and certain drugs. Each person’s cause of pulmonary hypertension will vary, depending on the type of PH they have:

Causes of Group 1 PH

Congenital heart disease.
Diet pills.
Genetic mutations.
HIV.
Liver disease.
Lupus.
Portal hypertension.
Pulmonary capillary hemangiomatosis.
Scleroderma.
Schistosomiasis.
Recreational drugs, such as methamphetamine.

Causes of Group 2 PH- Left-sided Heart Disease

Because the left and right side of the heart work together, a problem with either side will also affect the other side. Left-sided heart problems cause a chain reaction that affects the rest of your heart, pulmonary arteries and lungs.

Aortic valve disease.
Left-sided heart failure.
Left ventricular hypertrophy.
Mitral valve disease.

Causes of Group 3 PH- Lung Disease of Hypoxia

People with lung conditions are more at risk of pulmonary hypertension. Some people with left-sided heart disease also have a lung disease, including:

Chronic obstructive pulmonary disease (COPD).
Interstitial lung disease.
Obstructive sleep apnea.

Causes of Group 4 PH - Blockages in Your Lungs

This form of PH is typically caused by chronic thromboembolic pulmonary hypertension (CTEPH). This is a condition that involves blood clots and scarring in the arteries in your lungs.

A pulmonary embolism is a blood clot that travels from somewhere else in your body and ends up stuck in a blood vessel in your lungs. This can make it harder for blood to flow through your pulmonary arteries, making the blood pressure go up.

Causes of Group 5 PH - Other Disorders

There are many other conditions that cause PH that are not yet understood. However, there have been associations between certain conditions and PH, including:

Gaucher disease – a metabolic disorder that affects your organs and bones.
Kidney disease – problems with the functioning of your kidneys.
Sarcoidosis – inflammatory disease that affects your lungs and lymph glands.
Thyroid disease – affects your thyroid’s production of hormones.
Tumors – cancerous or benign tumors may put pressure on blood vessels in your lungs.

What are symptoms of pulmonary hypertension?

The first symptom people with pulmonary hypertension notice is shortness of breath during your daily activities, even as simple as climbing stairs or grocery shopping. Some people may not experience any other symptoms other than shortness of breath, or when they do, they are mild. PH symptoms get worse overtime, making it harder to live your daily life.

As PH progresses, you will find it more difficult to do daily activities without feeling short of breath. You may also experience:

Bluish color on your skin or lips.
Chest pain or pressure.
Dizziness or fainting.
Fatigue.
Feeling less hungry than usual.
Pain in the upper right side of your abdomen.
Racing heartbeat.
Swelling in your ankles, legs or abdomen.

How is pulmonary hypertension diagnosed?

Since the signs of pulmonary hypertension can be very similar to those of other conditions, it can be difficult to diagnose. Your provider will perform a physical examination and review your medical history. During the exam, they will check for signs of pulmonary hypertension as well as other heart or lung issues. This may include asking about your symptoms, listening to your heart and lungs, measuring your oxygen levels and blood pressure, and checking the size of the veins in your neck.

If your provider needs additional tests to support the diagnosis, they may order tests such as:

Right heart catheterization – measures the pressure inside your pulmonary arteries and checks how much blood your heart pumps per minute.
Doppler echocardiogram – uses soundwaves to show how your right ventricle is working and measures the blood flow through your heart valves.
Blood test – checks for issues such as organ function, hormone level and infections.
CT scan – looks for blood clots and other lung conditions.
X-ray – shows if your right ventricle or pulmonary arteries are bigger than they should be.
Six-minute walk test – shows how much exercise you can handle and how much oxygen is circulating in your blood as your exercise.

How is pulmonary hypertension treated?

Most cases of pulmonary HTN can’t be cured. Pulmonary hypertension treatment will depend on the type of PH you have and other medical conditions. Your provider will talk with you about treatment fit to your individual needs. Only two types of PH can be treated:

Treatment for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Anticoagulants to help prevent blood clots.
Balloon atrial septoplasty acts as a bridge that helps to keep you stable as you wait for a lung transplant.
Balloon pulmonary angioplasty uses a balloon to widen your pulmonary artery.
Pulmonary endarterectomy removes blood clots from your lungs.

Treatment for Pulmonary Arterial Hypertension (PAH)

Calcium channel blockers to help lower blood pressure in your pulmonary arteries and throughout your body.
Diuretics help your body clear out extra fluid.
Oxygen therapy helps to restore oxygen in your bloodstream.
Pulmonary vasodilators help your pulmonary arteries relax and open up better to improve blood flow and lowers the strain on your heart.

What are risk factors of pulmonary hypertension?

It’s not always possible to prevent pulmonary hypertension. It can happen to anyone, but there are certain risk factors that make you more likely to develop pulmonary HTN, including:

Family history of blood clots or pulmonary hypertension.
Living at high altitudes.
Smoking and using tobacco products.
Diet medications.
Prescription medications that treat cancer and depression.
Use of recreational drugs.

Other medical conditions also increase your risk of pulmonary hypertension:

Blood clots in your pulmonary arteries.
Connective tissue disorder.
Down syndrome.
Gaucher disease.
Heart disease.
HIV.
Liver disease.
Lung disease.