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Absent Pulmonary Valve—Child

Definition

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.

Heart Chambers and Valves

heart anatomy
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Blood Flow Through the Heart

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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.

Ventricular Septal Defect

Ventral septal defect
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Causes    TOP

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.

Risk Factors    TOP

Factors that may increase the risk of absent pulmonary valve may include:

  • Family history of congenital heart defect
  • Child has certain chromosomal disorders
  • Previous pregnancy with fetal heart abnormalities or miscarriage

Symptoms    TOP

Symptoms may include:

  • Blue or pale grayish skin color
  • Trouble breathing
  • Coughing
  • Poor feeding/poor weight gain

This condition can lead to heart failure. If your child has any of these symptoms, get medical care right away.

Diagnosis    TOP

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.

Treatment    TOP

Talk with the doctor about the best treatment plan for your child. Treatment options include:

Surgery

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.

Lifelong Monitoring

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.

Prevention    TOP

In most cases, there is no way to prevent absent pulmonary valve in your child. Getting proper prenatal care is always important.

RESOURCES:

American Heart Association
http://www.heart.org
Family Doctor—American Family Physician
http://familydoctor.org

CANADIAN RESOURCES:

Canadian Cardiovascular Society
http://www.ccs.ca
Heart and Stroke Foundation of Canada
http://www.heartandstroke.com

References:

Absent pulmonary valve. Helen B. Taussig Children’s Heart Center, Johns Hopkins University website. Available at:
...(Click grey area to select URL)
Updated May 16, 2011. Accessed November 7, 2014.
Evaluation of the infant for congenital heart disease (CHD). EBSCO DynaMed Plus website. Available at: http://www.dynamed.... Updated November 11, 2015. Accessed September 29, 2016.
Tetralogy of fallot in infants and children. EBSCO DynaMed Plus website. Available at: http://www.dynamed.... Updated January 25, 2016. Accessed September 29, 2016.
Last reviewed December 2015 by Kari Kassir, MD
Last Updated: 12/20/2014

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This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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