Absent Pulmonary Valve—Child
by Deanna M. Neff, MPH
An absent pulmonary valve is a rare heart defect.
In a normal heart, blood flows from the body into the right atrium and on to the right ventricle. Blood is then pumped out of the heart through the pulmonary artery to the lungs. The blood picks up fresh oxygen in the lungs. The blood returns to the left atrium of the heart and goes into the left ventricle. There it is pumped out through the aorta to deliver oxygen-rich blood to the rest of the body.
With this defect, the pulmonary valve does not develop properly. The opening where the valve should be is also narrowed. The defect causes the blood moving from the right ventricle to the arteries leading to the lungs to build up. This build-up causes swelling of these arteries that can put pressure on the air passages in the lungs.
The condition can be mild to severe. It usually occurs with other heart defects, like tetralogy of Fallot (a group of heart defects), or with an opening between the ventricles called a ventricular septal defect.
Absent pulmonary valve is a congenital defect. This means that the baby is born with it. It is not known exactly why some babies’ hearts develop abnormally.
Factors that may increase the risk of absent pulmonary valve may include:
Symptoms may include:
This condition can lead to heart failure. If your child has any of these symptoms, get medical care right away.
You will be asked about your child’s symptoms and medical history. A physical exam will be done.
Images may be taken of your child's bodily structures. This can be done with:
The electrical activity of your child's heart may be tested. This can be done with electrocardiogram.
Talk with the doctor about the best treatment plan for your child. Treatment options include:
Surgery aims to improve function and blood flow. In milder cases, closing the septal defect may be all that is needed. In other cases, surgery may be needed to repair the valve. A human or synthetic valve is used to replace the defective pulmonary valve.
After surgery, your child will need to have regular visits with a heart doctor. Your child may also need to take antibiotics before some medical or dental procedures to prevent a heart infection.
In most cases, there is no way to prevent absent pulmonary valve in your child. Getting proper prenatal care is always important.
American Heart Association
Family Doctor—American Family Physician
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
Absent pulmonary valve. Helen B. Taussig Children’s Heart Center, Johns Hopkins University website. Available at:
...(Click grey area to select URL)
Updated December 26, 2016. Accessed December 27, 2017.
Evaluation of the infant for congenital heart disease (CHD). EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated November 22, 2017. Accessed December 27, 2017.
Tetralogy of Fallot in infants and children. EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated June 19, 2017. Accessed December 27, 2017.
Last reviewed November 2018 by EBSCO Medical Review Board Kari Kassir, MD
Last Updated: 12/20/2014
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