by Neff DM

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
IMAGE
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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

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

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

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

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

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

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: http://www.pted.org/?id=absentpulmonaryvalve1. Updated May 16, 2011. Accessed November 7, 2014.

Evaluation of the infant for congenital heart disease (CHD). EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated September 16, 2014. Accessed November 7, 2014.

Tetralogy of fallot in infants and children. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated October 14, 2014. Accessed November 7, 2014.

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