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 partially anomalous pulmonary venous return (or “PAPVR”), which is also known as “partial anomalous pulmonary venous connections” (or “PAPVC”), we’re here to ease your concerns, answer your questions, and give your child the best possible chance for a healthy future.
Partially anomalous pulmonary venous return (PAPVR) is similar to total anomalous pulmonary venous connection (or “TAPVC”) in that it’s an abnormality of the connection between the pulmonary veins (which carry oxygenated blood from the lungs to the left side of the heart) and the left atrium (the upper left-hand chamber of the heart that receives oxygen-rich red blood from the lungs and then sends this blood to the left ventricle).
Usually, the pulmonary veins are all connected to the left atrium. When none of them are connected to the left atrium, it’s called “total anomalous pulmonary venous connection.” When some but not all of the pulmonary veins are connected properly, it’s called “partial anomalous pulmonary venous connection” (or “PAPVC”).
Partially anomalous pulmonary venous return (PAPVR) frequently happens in combination with other diagnoses, most often a sinus venosus atrial septal defect (ASD). PAPVC ranges from relatively harmless (as when it’s in association with a superior sinus venosus ASD, to more complicated, such as in a condition called, “Scimitar syndrome.” With Scimitar syndrome, the right pulmonary vein drains to the inferior vena cava (a large vein draining the lower body) rather than into the left atrium. Children with Scimitar syndrome usually have other abnormalities of the right lung, including a small right lung (called “hypoplasia”) and abnormal blood supply to the lung.
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:
In all forms of partially anomalous pulmonary venous return (PAPVR), oxygen-rich (“red”) blood returns to the right side of the heart. So children with the defect are not cyanotic (when the skin looks bluish in color because there’s not enough oxygen), but they have extra blood flow returning that makes the right side of the heart work harder and can eventually result in heart failure.
PAPVC is usually diagnosed when a caregiver hears a murmur while examining the child, although abnormalities on a routine chest X-ray or other imaging study may also suggest the diagnosis. At the Nemours Cardiac Center, we can confirm the diagnosis with an echocardiogram (or “echo”). 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 drainage isn’t clear from the echo, then we may need to perform additional tests, including cardiac catheterization, a CT scan or an MRI.
Most children with partially anomalous pulmonary venous return (PAPVR) need surgery to repair the defect. Usually, this is done at the time of diagnosis in older children. In some cases, when a child is diagnosed early in life, surgery may be delayed. But children usually have surgery before 4 or 5 years old. Repair depends on the exact type of abnormal drainage, but involves some combination of disconnecting and reconnecting the vein and/or using a patch (also called a “baffle”) to redirect blood from the right to the left side of the heart.
Following repair, most children won’t need any further surgical procedures. Occasionally, and depending on the exact type of abnormal drainage, additional procedures may be required to enlarge pulmonary veins that aren’t growing well. However, we expect children with repaired partially anomalous pulmonary venous return (PAPVR) to live just as long as any other child.
If your child has PAPVR, 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