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.
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.
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.
Factors that may increase the risk of absent pulmonary valve may include:
Symptoms may include:
This condition can lead to heart failure. If your child has any of these symptoms, get medical care right away.
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.
Talk with the doctor about the best treatment plan for your child. Treatment options include:
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.
American Heart Association
Family Doctor—American Family Physician
Canadian Cardiovascular Society
Heart and Stroke Foundation of Canada
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 Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T566082/Evaluation-of-the-infant-for-congenital-heart-disease-CHD. Updated November 11, 2015. Accessed September 29, 2016.
Tetralogy of fallot in infants and children. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T115349/Tetralogy-of-Fallot-in-infants-and-children. Updated January 25, 2016. Accessed September 29, 2016.
Last reviewed December 2015 by Kari Kassir, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.