Unroofed Coronary Sinus Syndrome

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

1600 Rockland Road, Wilmington, DE 19803 | Get Map & Directions »

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 unroofed coronary sinus syndrome, 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?

The coronary sinus is a vein that drains blood from the muscle of the heart itself to the right atrium (the upper chamber of the heart that drains blood from the body). The “roof” of the coronary sinus separates it from the left atrium (the upper chamber of the heart that drains blood from the lungs).  Occasionally, the sinus may be partially or completely “unroofed,” meaning there’s one or more connections between the coronary sinus (containing oxygen-poor blood returning from the heart muscle) and the left atrium (which normally receives oxygen-rich blood from the lungs).

Unroofed coronary sinus syndrome is usually associated with an atrial septal defect (or “ASD”), a hole between the right and left atria (the upper chambers of the heart). This results in oxygen-poor blood from the heart muscle mixing with the oxygen-rich blood from the lungs. When this happens, the oxygen levels in the blood moving from the left atrium to the ventricle and then out to the body are lowered.

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?

Unroofed coronary sinus syndrome may be diagnosed at any age (there are almost certainly adults walking around who’ve never been diagnosed). The defect is usually detected using an echocardiogram (or “echo”) after a child or adult is found to be bluish (or “cyanotic”) because of the lower oxygen levels. An echo is a completely safe and painless test that uses ultrasound (sound waves) to build a series of pictures of the heart.

How & When Is It Treated?

Whenever a child is diagnosed with unroofed coronary sinus syndrome, we recommend surgery to correct the defect. The surgical repair involves recreating the roof of the coronary sinus with a patch and separating the coronary sinus from the left atrium. At the same time, we close any associated atrial septal defect with a patch.

In newborns, an operation can safely be delayed to allow the baby to grow. If left untreated, though, children can develop complications because of the low oxygen levels and the presence of a hole between the right and left sides of the heart. But the results of surgical repair are excellent.

Learn More About Heart Surgery »

How Will It Affect My Child Long Term?

Most children won’t need additional surgery — and most will be able to function and exercise normally in the long-run.

If your child has unroofed coronary sinus syndrome, 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


+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 »