Babies who are born with a single-ventricle defect have only 1 pumping chamber (ventricle) in their heart that works, instead of the usual 2.

Single-ventricle defects are a group of complex and rare disorders. The affected ventricle may be smaller than the other, underdeveloped or missing a valve. This prevents it from pumping blood effectively to either the lungs or the body.

Single-ventricle defects are some of the most serious forms of congenital heart disease.

A wide range of cardiac conditions are considered “single-ventricle defects,” including:

Some type of single-ventricle defect affects 1 in every 20,000 newborns. These anomalies are usually diagnosed either during pregnancy, with a fetal echocardiogram, or right after birth, with an echocardiogram.

Children with single-ventricle defects have a series of operations during infancy and childhood. These surgeries change the circulation to an altered system, called the Fontan circulation. The majority of these children survive and go on to lead active lives.

Seattle Children’s Single Ventricle Program provides compassionate, comprehensive care for all types of single-ventricle defects, with particular emphasis on the vulnerable period between the first and second surgeries (also known as “interstage”).

What’s special about Seattle Children’s Single Ventricle Program?

  • We have the medical and surgical expertise to handle even the most complex cases – from prenatal diagnosis through treatment and lifelong follow-up care.
  • Our weekly interstage clinic brings together experts in cardiology, nutrition, social work, feeding therapy and neurodevelopment to support your child’s overall health between the first and second surgeries.
  • Seattle Children’s Hospital’s outcomes for surgeries to reconfigure the heart’s circulation system are among the best in the nation.
  • We’re committed to your child’s overall health and well-being. We’ll discuss treatments in ways you can understand and involve you in every decision.
  • U.S. News & World Report consistently ranks Seattle Children’s cardiology and heart surgery program as one of the best in the country.

Our program coordinates services including:

Prenatal diagnosis

Through our Prenatal Diagnosis and Treatment Program, we work with expectant parents to diagnose congenital heart defects during pregnancy. We provide thoughtful counseling and pregnancy management support. This enables both you and your child’s doctors to plan for the care that will be needed right after your baby’s birth.

Advanced imaging

Because single-ventricle anomalies are among the most complex congenital heart defects, it’s essential to get an accurate picture of your child’s unique anatomy.

Our heart doctors (cardiologists) and heart surgeons combine years of experience treating the most complex defects with advanced imaging techniques, including cardiac MRI (magnetic resonance imaging), echocardiography and 3D imaging. They’ll use these advanced imaging technologies to create a personalized repair plan that meets your child’s needs and to monitor your child’s progress.

Surgical expertise

Your baby will likely need several surgeries to improve their blood flow. The exact procedures and timing depend on your child’s diagnosis, symptoms and body. The surgeries are done in stages during the first few years of life.

Having experienced pediatric heart surgeons is of the utmost importance: doctors skilled enough to know not just how to operate, but when and what type of surgery is needed in your child’s particular situation. Each of our 4 heart surgeons is board certified in pediatric cardiac surgery. This means they have more than 12 years of training in complex newborn and infant procedures. Our surgeons are national leaders in the use of mechanical heart assist devices, as well as complex pediatric heart transplantation. Most importantly, our outcomes for surgeries to reconfigure the heart’s circulation system are among the best in the nation.

Cardiac catheterization and hybrid procedures

Heart doctors at Seattle Children’s are leaders in using new techniques, such as hybrid procedures, for babies with complex heart problems. This approach combines surgery and cardiac catheterization. Hybrid procedures can be life saving for babies who are too sick to go through open-heart surgery or too small for other treatments.

Single Ventricle Interstage Clinic

The first 6 months of life, the “interstage” period between the first and second surgeries, is a very vulnerable time for children with single-ventricle defects.

Our goal is to provide comprehensive care to improve growth and development and to prevent life-threatening events. Multiple specialists, including heart doctors (cardiologists), nutritionists, feeding therapists, social workers and neurodevelopmental pediatricians, work with you and your primary care provider to help your child be as healthy and active as possible.

Our weekly clinic includes:

  • Multidisciplinary care of single-ventricle patients from the first operation to the second. Your child’s care is coordinated by bringing multiple specialists to the same clinic appointment. This means fewer visits to the hospital.
  • Home monitoring of symptoms, oxygenation, feeding progress and weight. This ensures easy and early communication with the Heart Center and can help detect problems early. This is particularly important for families who live far from our Seattle campus.
  • Education for you and other family members involved in the care of your child
  • Clear communication between your child’s primary care provider and the specialists in the Heart Center

Advancing Treatment for Children

The Heart Center team at Seattle Children’s participates in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a project dedicated to improving the outcomes and quality of life for children with single-ventricle heart defects.

Read more about the Heart Center team’s research and advances.

Who’s on the team?

The Single Ventricle Program includes cardiologists, cardiac surgeons, nurse practitioners, nurses, nutritionists, physical/occupational/speech therapists and social workers. We work closely with you, your primary care physician and your primary cardiologist to provide comprehensive care for your child.

Cardiologists

Cardiac surgeons

Neurodevelopmental pediatrician

Program coordinator

  • Kendra Waldburger, RN, program coordinator

Contact Us

Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.