Overview

Pulmonary valve disease is a type of heart valve disease. It affects the valve between the heart's lower right chamber and the artery that delivers blood to the lungs. That artery is called the pulmonary artery. The valve is called the pulmonary valve.

A diseased or damaged pulmonary valve doesn't work the way it should. It changes how blood flows from the heart to the lungs.

Types of pulmonary valve disease include:

  • Pulmonary valve stenosis. The valve is narrowed. This reduces the blood flow from the heart to the pulmonary artery and lungs.
  • Pulmonary valve regurgitation. The flaps of the pulmonary valve don't close tightly. Blood moves backward into the right lower heart chamber, called the right ventricle.
  • Pulmonary atresia. This condition is present at birth. That means it is a congenital heart defect. The pulmonary valve isn't formed. Instead, a solid sheet of tissue blocks blood flow from the right side of the heart. Blood can't go to the lungs to pick up oxygen.

Symptoms

Symptoms of pulmonary valve disease depend on the specific valve condition and how much blood flow is changed or blocked. Some babies are born with pulmonary valve disease. Symptoms may be noticed soon after birth. But sometimes symptoms aren't seen until later in life.

In general, pulmonary valve disease symptoms may include:

  • Chest pain.
  • Shortness of breath at rest or when active or lying down.
  • Fatigue.
  • Fainting.

Babies born with pulmonary valve disease may have blue or gray skin due to low oxygen levels. Depending on skin color, these changes may be harder or easier to see.

When to see a doctor

Make an appointment for a health checkup if you or your child has:

  • Shortness of breath.
  • Chest pain.
  • Fainting.

Call 911 or your local emergency number if chest pain is sudden or can't be explained.

Quick diagnosis and treatment of pulmonary valve disease can help reduce the risk of complications.

Causes

Usually, pulmonary valve disease is caused by a heart condition that develops before birth, called a congenital heart defect. But pulmonary valve disease may happen later in life as a complication of another illness.

To understand how pulmonary valve disease happens, it might help to know how the valve works.

The pulmonary valve is like a one-way door from the lower right heart chamber, called the right ventricle, to the lungs. Blood usually flows from that chamber through the pulmonary valve and into the lungs. Blood gets oxygen in the lungs to take to the body.

When the pulmonary valve doesn't work as it should, the heart must work harder to pump blood to the lungs. The extra work can cause the right ventricle to become thick.

Risk factors

Things that can raise the risk of pulmonary valve disease are:

  • A heart condition that you're born with, also called a congenital heart defect. Some congenital heart defects affect the shape of the pulmonary valve and how it works.
  • Carcinoid syndrome. This condition happens when a rare cancerous tumor sends chemicals into the bloodstream. It can cause carcinoid heart disease, which commonly damages the pulmonary and tricuspid valves.
  • Chest injury. An injury to the chest, such as from a car accident, may cause damage that leads to pulmonary valve regurgitation.
  • German measles, also called rubella. Having German measles during pregnancy increases the risk of pulmonary valve stenosis in the baby.
  • Infection of the lining of the heart and heart valves, also called endocarditis. This condition can damage the pulmonary valve. IV drug misuse makes a person more likely to get endocarditis.
  • Noonan syndrome. This condition is caused by changes in genes. Pulmonary valve stenosis is a common heart complication seen in people with Noonan syndrome.

Feb. 25, 2025

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