Absent Pulmonary Valve—Child
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 and returns to the left atrium and goes into the left ventricle. From there, it is pumped out through the aorta to deliver oxygen-rich blood to the rest of the body.
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An absent pulmonary valve is one that is missing or poorly formed. 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 causes swelling of these arteries that can put pressure on the air passages in the lungs.
This problem 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 .
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A baby is born with this heart defect. It is not known why this happens.
Things that may raise the risk of this problem are:
- A family history of heart defects at birth
- Certain chromosomal disorders
- Prior pregnancy with fetal heart abnormalities or miscarriage
Problems may be:
- Blue or pale grayish skin color
- Trouble breathing
- Poor feeding
- Poor weight gain
The doctor will ask about your child’s symptoms and health history. A physical exam will be done.
Pictures may be taken of your child's heart. This can be done with:
Your child's heart function may be tested. This can be done with:
Surgery will be needed to improve heart function and blood flow. Choices are:
- Closing a mild septal defect
- Repairing the valve
- Replacing the valve with a donor or synthetic valve
Lifelong heart monitoring will be needed after treatment.
There are no known guidelines to prevent an absent pulmonary valve.
- Absent pulmonary valve. Helen B. Taussig Children’s Heart Center, Johns Hopkins University website. Available at: http://www.pted.org/?id=absentpulmonaryvalve1.
- Evaluation of the infant for congenital heart disease (CHD). EBSCO DynaMed website. Available at: https://www.dynamed.com/evaluation/evaluation-of-the-infant-for-congenital-heart-disease-chd.
- Tetralogy of Fallot in infants and children. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/tetralogy-of-fallot-in-infants-and-children.
- Kari Kuenn, MD
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