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Absent Pulmonary Valve—Child by Deanna M. Neff, MPH

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

Blood Flow Through the Heart

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

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

Some risk factors for congenital heart diseases like 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

The doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:

Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
Chest x-ray —an imaging test that uses low amounts of radiation to create an image of the chest
Electrocardiogram —a test that measures the electrical activity of the heart
MRI scan —a test that uses magnetic waves to make pictures of structures inside the chest
CT scan —a type of x-ray that uses a computer to make pictures of structures inside the chest
Cardiac catheterization —a test that uses a catheter (tube) and x-ray machine to assess the heart and its blood supply

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 Family Physician http://www.aafp.org/
American Heart Association http://www.americanheart.org/

CANADIAN RESOURCES:

Canadian Cardiovascular Society http://www.ccs.ca/
Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

References:

American Heart Association. How your cardiologist diagnoses heart defects. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=152 . Updated May 19, 2009. Accessed July 7, 2010.
Cove Point Foundation. Absent pulmonary valve. Helen B. Taussig Children’s Heart Center, Johns Hopkins University website. Available at: http://www.pted.org/?id=absentpulmonaryvalve1 . Accessed July 7, 2010.
DynaMed Editors. Evaluation of the infant for congenital heart disease. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated April 14, 2010. Accessed July 7, 2010.
DynaMed Editors. Tetralogy of fallot. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated June 10, 2010. Accessed July 20, 2010.

Last reviewed June 2012 by Kari Kassir, MD

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