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Pulmonary Artery Sling
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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 pulmonary artery sling, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Pulmonary artery sling (also known as an “aberrant left pulmonary artery”) is a rare condition in which the left pulmonary artery (which receives blood from the right ventricle and carries it to the left lung) had an abnormal origin and course as it travels to the left lung. Instead of branching off from the main pulmonary artery, as it usually does, it forms as a branch off of the right pulmonary artery. It then passes between the trachea (or windpipe, which carries inhaled air from the throat to the lungs) and esophagus (a muscular tube that carries food and liquids from the mouth to the stomach) to reach the left lung.
Although pulmonary artery sling doesn’t block blood flow to the lungs, it’s often associated with severe tracheobronchial anomalies (problems with the trachea and bronchi, the air tubes at the bottom of the trachea). The trachea may develop abnormally and often has an area or areas of narrowing (this is called “tracheal stenosis”).
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:
The symptoms of a pulmonary artery sling are related to compression (or squeezing) of the trachea and esophagus by the abnormal pulmonary artery. Most children with this anomaly will have symptoms shortly after birth, including wheezing and coughing (similar to children with asthma). Some newborns won’t be able to breathe on their own and will need a ventilator to help them breath until the sling is repaired.
All children with symptoms and the presence of a pulmonary artery sling need surgery. Usually, the repair involves disconnecting the abnormal left pulmonary artery and returning it to a more normal position where it won’t cause compression of the trachea or esophagus. In some children, the trachea may also need to be repaired at the same time. The outcomes of surgery depend primarily on the severity and extent of the tracheal narrowing.
Children rarely require additional operations on the pulmonary artery itself. But when the tracheal narrowing is severe or extends a long distance along the trachea, reoperations to enlarge the trachea are more common. Children with severe tracheobronchial anomalies may have ongoing symptoms and require multiple operations over their lifetime.
If your child has pulmonary artery sling, 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.
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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