Pulmonary hypertension facts for kids

Quick facts for kids Pulmonary hypertension
Synonyms	Ayerza syndrome
Pulmonary hypertension affects the arteries in the lungs and the right side of the heart.
Symptoms	Shortness of breath, tiredness, chest pain, fast heartbeat
Usual onset	Usually between 20 and 60 years old
Duration	Long term
Causes	Often unknown
Risk factors	Family history, blood clots in the lungs, sickle cell disease, COPD, sleep apnea, living at high altitudes
Diagnostic method	Based on tests that rule out other causes
Treatment	Supportive care, various medications, lung transplantation
Medication	Various medications that help the heart and lungs
Frequency	About 1,000 new cases a year in the U.S.

Pulmonary hypertension (PH) is a condition where the blood pressure is too high in the blood vessels that go from the heart to the lungs. These blood vessels are called the pulmonary arteries.

When the pressure is high, the heart has to work much harder to pump blood to the lungs. This can cause symptoms like shortness of breath, feeling very tired, chest pain, and a fast heartbeat. The condition can make it difficult to exercise or do daily activities. PH usually develops slowly over time.

While there is no cure for pulmonary hypertension, there are many treatments that can help manage the symptoms and improve a person's quality of life. Scientists are always working on finding new and better treatments.

What Are the Signs and Symptoms?

People with pulmonary hypertension may experience a variety of symptoms. These can include:

What Causes Pulmonary Hypertension?

Pulmonary hypertension is a complex condition that can have many different causes. To make it easier to understand, doctors often sort it into five main groups.

• Group 1: This type is caused by the narrowing of the small arteries in the lungs. Often, the exact cause is unknown (this is called idiopathic). Sometimes, it can be passed down through families (heritable) or be linked to other health conditions.
• Group 2: This is the most common type and is caused by problems with the left side of the heart. If the left side of the heart can't pump blood properly, it can cause a "backup" of pressure in the lungs.
• Group 3: This type is related to lung diseases like COPD or conditions that cause low oxygen levels in the body (hypoxia), such as living at a very high altitude.
• Group 4: This type is caused by long-term blood clots in the lungs. These clots can block the arteries and increase the pressure.
• Group 5: This group includes PH caused by a mix of other health problems, such as blood disorders or certain metabolic disorders.

How Does It Affect the Body?

Animation showing the heart. The right ventricle (on the left side of the image) pumps blood to the lungs.

A microscope view of a lung artery in someone with pulmonary hypertension. The wall of the artery is much thicker than normal.

The main problem in pulmonary hypertension is the narrowing of the blood vessels in the lungs. This makes it much harder for the heart to push blood through them. Think of it like trying to blow air through a very narrow straw compared to a wide one—it takes a lot more effort.

Over time, this extra work puts a big strain on the right side of the heart, which is responsible for pumping blood to the lungs. The heart muscle can get thicker and larger to try to keep up, a condition called hypertrophy.

Eventually, the right side of the heart can become too weak to pump enough blood to the lungs. This is known as right-sided heart failure. When this happens, the body doesn't get all the oxygen-rich blood it needs, which is why people with PH often feel tired and out of breath.

How Do Doctors Diagnose It?

A CT scan showing a widened pulmonary artery, which can be a sign of pulmonary hypertension.

Diagnosing pulmonary hypertension involves several steps to rule out other conditions and find the cause.

Physical Exam

A doctor will first perform a physical exam, listen to the heart and lungs, and ask about symptoms. They will also ask about the person's family medical history to see if the condition might run in the family.

Heart and Lung Tests

Several tests can help doctors see how the heart and lungs are working:

• Echocardiogram: This is an ultrasound of the heart. It uses sound waves to create a picture of the heart as it beats. It can show if the right side of the heart is enlarged and can estimate the pressure in the pulmonary arteries.
• Electrocardiogram (ECG): This test records the heart's electrical activity and can show signs of strain on the right side of the heart.
• Chest X-ray: An X-ray can show if the pulmonary arteries or the right side of the heart are larger than normal.
• CT scan: This provides a more detailed picture of the lungs and blood vessels.
• Breathing Tests: These tests, called pulmonary function tests, measure how well the lungs are working.

Right Heart Catheterization

A diagram of a pulmonary artery catheter, a tool used to measure pressure inside the heart and lungs.

While the tests above can suggest PH, the most accurate way to diagnose it is with a right heart catheterization. In this procedure, a doctor guides a very thin, flexible tube (a catheter) through a blood vessel into the right side of the heart and into the pulmonary artery. This allows them to measure the blood pressure directly.

How Is It Treated?

The goal of treatment is to manage symptoms, slow down the disease, and improve a person's ability to be active. Treatment depends on the type and seriousness of the PH.

Medications

There are several types of medicines that can help treat pulmonary hypertension:

• Vasodilators: These medicines help relax and open up the narrowed blood vessels in the lungs, which lowers the blood pressure.
• Endothelin Receptor Antagonists: These drugs help block a substance in the body that causes blood vessels to narrow.
• Other Medications: Doctors may also prescribe diuretics (water pills) to reduce swelling or anticoagulants (blood thinners) to help prevent blood clots.

Therapies and Support

• Oxygen Therapy: Some people with PH need to breathe in extra oxygen through a mask or nasal prongs to help raise the oxygen levels in their blood.
• Exercise: Under a doctor's guidance, gentle exercise like walking can help improve strength and breathing.

Surgical Procedures

In very severe cases, when medications are not working well enough, doctors might suggest surgery.

• Atrial septostomy: A procedure that creates a small hole between the heart's upper chambers to relieve pressure on the right side of the heart.
• Lung transplantation: Replacing one or both lungs with healthy donor lungs may be an option for some people.
• Pulmonary Thromboendarterectomy (PTE): For people with PH caused by blood clots (Group 4), this surgery removes the old clots from the pulmonary arteries.

Living with Pulmonary Hypertension

Pulmonary hypertension is a serious, long-term condition. However, thanks to new treatments developed over the last few decades, people with PH are living longer and more active lives than ever before.

It is a rare disease, and it tends to affect females more often than males. Managing the condition often involves regular doctor visits and carefully following a treatment plan. Research is ongoing to find even better treatments and, one day, a cure.

Notable People with the Condition

• Elaine Kaufman, an American restaurant owner
• Ina Balin, an American actress
• Chloe Temtchine, an American singer-songwriter
• Natalie Cole, a famous American singer

Images for kids

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  Diagram showing pathways in the body that are affected by pulmonary hypertension.

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  A phonocardiogram, which records heart sounds.

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  An image from an echocardiogram showing a heart valve issue.

See also

In Spanish: Hipertensión pulmonar para niños

• Pulmonary Hypertension Association
• Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)