Anomalous Origin of the Right or Left Pulmonary Artery of the Aorta

Nemours Cardiac Center at Nemours/Alfred I. duPont Hospital for Children

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Children’s heart conditions can’t be prevented, but a lot can be done to improve and often completely repair their hearts at any age. Thanks to advanced technology and the pediatric heart experts at the Nemours Cardiac Center (based at Nemours/Alfred I. duPont Hospital for Children), most children born with a heart problem — even newborns only hours or days old — can be quickly diagnosed and treated right when it matters the most. At the Cardiac Center, we specialize in early detection and repair of congenital heart defects (also often called, “congenital heart disease”).

If your child’s been diagnosed with anomalous origin of the right or left pulmonary artery off the aorta, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.

 
What Is It?

Normally, blood leaves the heart from the right ventricle through the pulmonary artery. The main pulmonary artery then branches into a right and left pulmonary artery that carry the blood to the right and left lungs. Sometimes, instead of connecting the main pulmonary artery to a lung, one of those branches makes a connection between the aorta and the lung. This is called “anomalous origin of the right or left pulmonary artery off the aorta.” (“Anomalous” means abnormal.)

The result of this abnormal connection is that oxygenated blood from the left ventricle circles back through the lungs, rather than travelling out to the body. This leads to at least two problems:

  1. The heart has to pump more blood than normal because some of it’s circling back to the lungs.
  2. The extra blood going to the lungs can damage the blood vessels and increase blood pressure in the lungs (this is called, “pulmonary hypertension”).

How Does the Heart Normally Work?

When your child has a congenital heart defect, there’s usually something wrong with the structure of the heart. In order to understand your child’s condition, it can help to know how the heart should work normally.
Learn More About Normal Cardiac Anatomy »

Nemours’ experts at KidsHealth.org also offer these helpful resources to help both you and your child understand how the heart works:

 
How & When Is It Diagnosed?

Anomalous origin of the right or left pulmonary artery off the aorta is usually diagnosed at birth or soon after. Children may have symptoms that their heart is failing (this is called, “heart failure”) including:

  • poor growth
  • rapid breathing
  • rapid heart rate

Learn More
 
How & When Is It Treated?

To repair an anomalous origin of the right or left pulmonary artery off the aorta, we perform open-heart surgery using a patch soon after diagnosis. The patch may be one of the following:

  • from the child’s own tissue (usually the sac around the heart, called the “pericardium”)
  • tissue from the sac around the heart of a cow (called the “bovine pericardium”)
  • human tissue (called a “homograft”)
  • artificial tissue such as polytetrafluoroethylene (“PTFE” or Gore-Tex)
  • woven tissue (Dacron)

All of these options are safe and work well for the children we treat. Your child’s Nemours Cardiac Center heart surgeon will usually determine, in the operating room, which kind of patch will work best for your child.

Learn More About Heart Surgery »

 
How Will It Affect My Child Long Term?

Surgery makes the blood flow normally through the heart. But because the original connection of the pulmonary artery wasn’t normal, as children grow, we’ll need to monitor the pulmonary artery’s size and growth. Children may require additional procedures if the abnormal pulmonary artery growth doesn’t grow as they grow.

If your child has anomalous origin of the right or left pulmonary artery off the aorta, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care. Our goal is to guide your family, from start to finish, through your child’s heart defect journey — and to help your child live the healthiest, most fulfilling life possible.

Learn More About Congenital Heart Defects »

Why Choose Us

From our outcomes to our family-centered care, find out all of the reasons why your child’s heart will be in good hands at the Nemours Cardiac Center. Learn More »

Make an Appointment

(800) 416-4441

Insurance We Accept »


Information for Patients

Outpatient Services and Inpatient Units: (302) 651-6660

After 5 p.m. and Weekends:

Cardiac Intensive Care Unit: (302) 651-6644
General Inpatient Unit, 2B: (302) 651-6690


International
Patients

+1 (302) 651-4993

Stories From the Heart

Meet children treated in the Nemours Cardiac Center who know what it’s like to live with a heart problem.

Read Their Inspiring Stories »