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Lahey Health is now part of Beth Israel Lahey Health

by EBSCO Medical Review Board

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
Copyright © Nucleus Medical Media, Inc.

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 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 .

Ventricular Septal Defect
Ventral septal defect
Copyright © Nucleus Medical Media, Inc.

Causes

This problem is a congenital defect. This means that the baby is born with it. It is not known exactly why this happens.

Risk Factors

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

Symptoms

Problems may be:

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

Diagnosis

The doctor will ask about your child’s symptoms and health history. A physical exam will be done.

Images may be taken of your child. This can be done with:

Your child's heart function may be tested. This can be done with:

Treatment

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.

Prevention

There are no known guidelines to prevent this health problem.

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.ca 

References

Absent pulmonary valve. Helen B. Taussig Children’s Heart Center, Johns Hopkins University website. Available at: http://www.pted.org/?id=absentpulmonaryvalve1. Accessed March 10, 2021.

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. Accessed March 10, 2021.

Silberbach M, Hannon D. Presentation of congenital heart disease in the neonate and young infant. Pediatr Rev. 2007 Apr;28(4):123-131.

Tetralogy of Fallot in infants and children. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/tetralogy-of-fallot-in-infants-and-children. Accessed March 10, 2021.

Revision Information

  • Reviewer: EBSCO Medical Review Board Kari Kassir, MD
  • Review Date: 12/2020
  • Update Date: 03/10/2021