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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 cor triatriatum, we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Cor triatriatum is an abnormality in the connection from the pulmonary veins draining blood from the lungs into the left atrium (where the blood gets from the lungs to the heart and is then pumped out to the body). Instead of the veins coming together directly into the left atrium, as they should, they drain into an extra chamber located behind the left atrium. There can be one or more connections between this chamber and the left atrium, but the connection is narrow and causes obstruction to blood flowing from the lungs to the left atrium and heart.
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 timing of symptoms and diagnosis in cor triatriatum are determined by the relative size of the connection between the extra chamber and the left atrium — in most cases that connection is small. Children begin to have symptoms in the newborn period as their lungs become filled with fluid (or “congested”) because of the obstruction to blood leaving the lungs for the heart. Rarely, the connection may be big — when this happens, children may grow to adulthood without being diagnosed.
An echocardiogram (or “echo”) is usually the only test necessary to diagnose cor triatriatum, although sometimes children may need additional studies. An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.
If the connection is narrowed, cor triatriatum needs to be repaired when it’s diagnosed. Children diagnosed with severe obstruction in the newborn period may need emergency surgery. Occasionally, as with total anomalous pulmonary venous connection, newborns may become so sick so fast that it’s better to use extra-corporeal membrane oxygenation (or “ECMO”) to help very sick infants recover so that they can tolerate a heart operation. (ECMO is a life-saving technique that replaces the function of the heart and lungs, allowing the child’s heart and lungs to rest and the organs to recuperate. During ECMO treatment, the heart continues to beat, but a mechanical pump does most of the work.)
In children with less severe obstruction, surgical repair can safely be delayed, but most children will need an operation.
Following repair, most children won’t need any further surgical procedures. Occasionally, additional procedures may be required to enlarge pulmonary veins that are not growing well. However, we expect children with repaired cor triatriatum to have a normal life expectancy.
If your child has cor triatriatum, 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