Single Ventricle Defects | Overview

LIke ThisLIke ThisLIke ThisLIke ThisLIke This

Contact the Complex Biventricular Repair Program

What is a single ventricle defect?

A single ventricle defect is a type of heart defect that a child is born with. It occurs when one of the two pumping chambers in the heart, called ventricles, isn’t large enough or strong enough to work correctly. In some cases, the chamber might be missing a valve.

Single ventricle heart defects can cause children to become cyanotic (turn a blue color), since a mixture of oxygen-poor (blue) and oxygen-rich (red) blood vessels leaves the heart and goes to the body. Just how much oxygen or how little oxygen depends on the type, location and severity of the defect. Some children will only be mildly cyanotic, while others won’t have enough oxygen in the blood to meet the body's needs and will need early treatment.

Single ventricle defects are rare, affecting only about five out of 100,000 newborns. They are also one of the most complex heart problems, usually requiring at least one surgery.

There are several types of single ventricle defects. These include:

The uniquely complex anatomy of the heart and its chambers make each ventricle defect different.

Care for single ventricle defects

At Boston Children’s Hospital, our experienced surgeons in the Department of Cardiac Surgery treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent — among the highest in the nation among large pediatric cardiac centers.

Boston Children’s Congenital Heart Valve Program cares for children with congenital heart defects that involve absent or malfunctioning heart valves. Our surgeons have a strong record of excellence in heart valve repair and replacement, including minimally invasive techniques.

Babies with single ventricle defects usually need emergency treatment. Our cardiac intensive care unit (CICU), cardiac catheterization (cath) labs and operating rooms deal with the urgent needs of our smallest patients 24/7. The Heart Center’s CICU was one of the first such units created anywhere, and professionals from many countries visit our CICU year-round to learn the advanced techniques of post-operative care.

At Boston Children’s, we provide families with a wealth of information, resources, programs and support before, during and after your child’s treatment. With our compassionate, family-centered approach to expert treatment and care, you and your child are in the best possible hands.

For visual and audio information on some congenital heart defects, visit Boston Children’s cardiovascular Multimedia Library.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944

Close