Meet Michael, who was 6 weeks old when he was diagnosed with aortic stenosis and treated by the pediatric heart experts at the Nemours Cardiac Center.
Aortopulmonary (AP) Window
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 aortopulmonary (or “AP”) window, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Aortopulmonary (AP) window is a rare condition (occurring in about 1 in 10,000 children) in which there’s an abnormal connection between the aorta and the pulmonary artery immediately after the blood leaves the heart. The abnormal connection allows oxygen-rich blood to travel from the aorta (the blood vessel that delivers oxygen-rich blood from the left ventricle to the body) into the pulmonary artery (the large artery that receives oxygen-poor blood from the right ventricle and carries it to the lungs).
This leads to at least two problems:
- The heart has to pump more blood than normal because some of it’s going in a circle back to the lungs (this is called a “volume load”).
- All of the extra blood going to the lungs can damage the blood vessels in the lungs and cause high 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:
Because of the location of the abnormal connection, there’s too much blood flow to the lungs with aortopulmonary (AP) window. Children usually have symptoms early in life indicating that their heart is failing (this is called “heart failure”), including:
- poor growth
- rapid breathing
- rapid heart rate
Your child’s doctor or nurse may hear a murmur during physical examination, prompting an echocardiogram (or “echo”) — a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart. We can usually make the diagnosis through an echo, but we may also perform additional imaging studies (including cardiac catheterization, CT scanning or MRI).
The large amount of extra blood flow to the lungs can cause permanent damage to both the heart and lungs, so we usually perform surgery to fix aortopulmonary (AP) window in the first few months after birth.
Repairing this defect generally requires open-heart surgery in order to ensure that the connection is closed and both the pulmonary valve and aortic valve continue to function well. Surgical closure primarily consists of identifying the abnormal connection and closing it with a patch.
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.
In certain cases, we may close the connection using interventional catheterization techniques. Catheterization is performed by inserting a thin plastic, flexible tube (called a “catheter”) into an artery and vein that lead to the heart. Cardiac catheterization is done under sedation with local anesthesia or under general anesthesia, depending on your child’s age and condition.
Surgery to repair the abnormal connection in aortopulmonary (AP) window creates normal circulation of blood through the heart and lungs. A few children won’t need any further operations, although occasionally growth may be restricted in either the pulmonary artery or the aorta in the region of the abnormal connection. Children may need additional procedures to ensure that there’s enough blood flow to the lungs.
If your child has aortopulmonary (AP) window, know that at the Nemours Cardiac Center we’re here to give your child the very best, most comprehensive and compassionate care possible. 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.
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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