Single Ventricle Program

What is the Single Ventricle Program?

Seattle Children’s Single Ventricle Program provides compassionate, comprehensive care for children with all types of single-ventricle defects. Our skilled team tailors treatment to your child. 

If your baby is diagnosed during pregnancy, experts at the Fetal Care and Treatment Center will assess your baby’s health and help you prepare for their birth. After your child is born, our multidisciplinary team will evaluate your baby’s heart and any related conditions to make a treatment plan specific to your child. In your baby’s first days of life and during childhood, we provide expert care, including complex heart surgeries and less invasive cardiac catheterization procedures to repair the defect.   

We have a strong focus on caring for babies during the critical period between their first and second surgeries (known as “interstage”), partnering with our regional clinics and providers. As your child grows, we provide coordinated care through Seattle Children’s Fontan Clinic for children and young adults who have had a Fontan procedure to improve their blood flow.

What is a single ventricle heart defect?

Babies born with a single-ventricle defect have only 1 pumping chamber (ventricle) in their heart that works, instead of the usual 2. The ventricle that does not work may be smaller than the other, underdeveloped or missing a heart valve. This keeps it from pumping blood well to either the lungs or the body.

Together, single-ventricle heart defects affect about 1 in every 2,000 children. This condition is also called single ventricle congenital heart disease.

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

With the right care, children with single-ventricle defects go on to lead active lives.

Conditions We Treat

We treat a wide range of single-ventricle defects, including:

• Double-inlet left ventricle (DILV)
• Hypoplastic left heart syndrome (HLHS)
• Unbalanced atrioventricular septal defect
• Tricuspid atresia, sometimes called hypoplastic right heart syndrome (HRHS)
• Severe Ebstein’s anomaly
• Pulmonary atresia with intact ventricular septum (PA/IVS)
• Heterotaxy  

"Single-ventricle anomalies are among the most complex congenital heart defects.

Our team works to support your child's overall health between the first and second surgeries, which can be a fragile time requiring close medical attention.

Our weekly interstage clinic brings together experts in cardiology, nutrition, social work, feeding therapy and neurodevelopment to support your child and family."

— Dr. Matt Files, director, Single Ventricle Program

What's special about Seattle Children's care for children with single ventricle defects?

Seattle Children has the medical and surgical expertise to handle even the most complex single-ventricle defects – from fetal diagnosis through treatment and long-term follow-up care. During pregnancy, our Fetal Care and Treatment Center team works with you to assess your baby’s health and prepare for their birth and care. We are experienced in the intensive medical treatment many babies need in their first days of life, and the complex surgeries during childhood. Our heart surgery survival rates are among the best in the nation, year after year. We closely monitor and care for your child during the critical period between the first and second surgeries (“interstage”). After Fontan surgery to change blood flow, our Fontan Clinic provides comprehensive care until your child reaches adulthood. We will help you transition to a cardiologist who specializes in care for adult patients with congenital heart disease. 

A dedicated team of experts

• The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. In the past 5 years, we have treated more than 200 newborns with single-ventricle defects.
• Our doctors and surgeons are experts in the treatments your child may need. These may include surgery to change the blood flow in your child’s heart, a hybrid procedure or a heart transplant.
• Our 4 pediatric cardiac surgeons perform more than 500 procedures yearly. Our survival rates for babies we treat are among the best in the nation year after year. See our outcomes for surgeries related to single-ventricle defects.
• Children who have heart surgery receive expert care from teams experienced in providing pediatric cardiac anesthesia and Cardiac Intensive Care for children. 
• To help your child be as healthy and active as possible between surgeries, our weekly Single Ventricle Interstage Clinic brings together specialists in cardiology, nutrition, social work, feeding therapy and neurodevelopment.
• For children who need a heart transplant, survival rates for our patients are among the best in the nation.
• Your child’s team includes experts from other areas of Seattle Children’s based on their needs, like doctors who specialize in newborns (neonatologists) or lung health (pulmonologists).

Care from fetal diagnosis through young adulthood

• If your developing baby is diagnosed with a single-ventricle defect before birth, Seattle Children’s Fetal Care and Treatment team works closely with you to plan and prepare for any care your baby may need.
• We work closely with your birth hospital and are national leaders in safely moving fragile newborns. 
• Our Level 4 (IV) Neonatal Intensive Care Unit (NICU) team is skilled and equipped to care for the sickest newborns.
• Your child’s treatment plan is custom-made. We plan and carry out their treatment based on the specific details of their heart defect. We closely check your child’s needs to make sure they get the care that is right for them at every age.
• If your child has a Fontan procedure, the multidisciplinary team in our Fontan Clinic provides coordinated care through childhood and young adulthood.
• We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.

Support for your whole family

• Whatever type of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
• Many of our single-ventricle patients come from outside the Seattle area. We know you may be away from your home, community and usual support systems when you are here for appointments or surgeries. We are here to help you and your whole family during this time.
• Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
• Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make the journey as smooth as possible.
• Read more about the supportive care we offer.

Advancing treatment for children

• The Heart Center team at Seattle Children’s takes part in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). This group works to improve the outcomes and quality of life for all children with single-ventricle heart defects. The NPC-QIC also offers resources, support and information for families.
• Read more about the Heart Center team’s research and advances.
• Learn about participating in clinical trials at Seattle Children’s.

“I contacted Seattle Children’s [for a second opinion], and it was there that I learned a lot more about my baby’s diagnosis. They made me feel more at ease.” – Sarah Ouellette, whose son Greyson was diagnosed with pulmonary atresia before birth, giving his family and his Seattle Children’s team time to plan and prepare

Single Ventricle Program Services and Treatments

Our experienced team tailors a treatment plan for your child to provide the best care for their specific defect and any related conditions. We provide care from fetal diagnosis until your child reaches adulthood. 

Fetal diagnosis 

Doctors usually diagnose single-ventricle defects during pregnancy with a fetal echocardiogram or right after birth with an echocardiogram.

Through our Fetal Care and Treatment Center, we work with parents to diagnose congenital heart defects during pregnancy. We provide thoughtful counseling and pregnancy management support. This lets you and your child’s doctors plan for the care your baby will need right after birth.

Advanced imaging 

Because these defects are so complex, your child’s care team needs to get an accurate picture of your child’s heart. Our heart doctors (cardiologists) and heart surgeons use advanced imaging techniques to do this. We use methods like cardiac MRI (magnetic resonance imaging), echocardiography and 3-D imaging. These let us make a personalized repair plan for your child and check your child’s progress.

Surgical expertise 

Your baby will likely need a series of surgeries during infancy and childhood to improve their blood flow. The exact procedures and timing depend on your child’s needs. We take into account their diagnosis, symptoms and body. 

In the first weeks of life, most children have a complex surgery called the Norwood procedure. The second stage, called the Glenn operation, is most often done by the time a child is 6 months old. Between 3 to 5 years of age, children have a Fontan procedure. These surgeries change their blood flow to a system called the Fontan circulation. 

Whenever possible, we do less invasive cardiac catheterization procedures to repair certain defects, such as patent ductus arteriosus (PDA). The doctor uses a small, thin tube (catheter), inserted through a blood vessel in your child’s leg. 

Having experienced pediatric heart surgeons is very important. Seattle Children’s heart surgeons know which type of surgery your child needs and are skilled in the complex procedures. Each has more than 12 years of training, including advanced fellowship training in congenital heart surgery. 

They are national leaders in the use of mechanical heart assist devices, including extracorporeal life support, as well as complex pediatric heart transplantation. Our survival rates for surgeries to change blood flow in the heart are among the best in the nation.

Cardiac catheterization and hybrid procedures 

Heart doctors at Seattle Children’s are leaders in using advanced techniques, such as hybrid procedures and patent ductus arteriosus (PDA) stenting, for babies with complex heart problems. Hybrid procedures combine surgery and cardiac catheterization. 

This approach can be lifesaving for babies who are too sick to go through open-heart surgery or too small for other treatments. Children have less pain and recover faster with catheterization procedures than with surgery for the same conditions. Our goal is to get the best results for your child in a way that is easiest on their body.

Single Ventricle Interstage Clinic 

The time between your child’s first and second surgeries is called the “interstage” period. During this time – the first 6 months of life - babies with single-ventricle defects are at risk for serious health problems from common childhood illnesses, like colds. They may have trouble feeding and growing.

Our clinic provides comprehensive care between surgeries. The focus is to improve your child’s growth and development and to prevent life-threatening events. A team of specialists works with you and your child’s primary care provider to help your child be as healthy and active as possible.

Our weekly clinic includes:

• Team-based (multidisciplinary) care. Your child’s team includes heart doctors (cardiologists), registered dietitian nutritionists, feeding therapists, social workers, neurodevelopmental pediatricians and others. We bring specialists together at the same clinic appointment. This means fewer visits to the hospital for you.
• Home monitoring. We track your child’s symptoms, oxygenation, feeding progress and weight. This makes it easy for your child’s Heart Center team to get important details and can help detect problems early. It helps all families, especially those who live far from our Seattle campus.
• Education. We answer your questions and make sure you and your family understand all you need to know about the care of your child.
• Clear communication. To make care as seamless as possible, specialists in the Heart Center keep in close touch with your child’s primary care provider.

Fontan Clinic

Your child will need ongoing care after their third surgery --the Fontan procedure -- to change their blood flow. Children and young adults with Fontan circulation are at risk for medical issues with their heart, liver, lungs, kidneys and other organs. Our multidisciplinary Fontan Clinic provides coordinated, team-based care to prevent and treat conditions that can happen as your child grows. 

Scheduling an Appointment With the Single Ventricle Program

• If you would like a referral to the Single Ventricle Program, talk to your child’s primary care provider.
• How to schedule an appointment at Seattle Children’s.
• If you already have an appointment, learn more about how to prepare.
• Learn about Heart Center resources such as useful links, videos and recommended reading for you and your family.

Who’s on the team?

The Single Ventricle Program includes cardiologists, heart surgeons, nurse practitioners, nurses, registered dietitian nutritionists, physical therapists, occupational therapists, speech and language pathologists and social workers. We work closely with you, your child’s primary care provider and your primary cardiologist to provide comprehensive care for your child.

Contact Us

Contact the Heart Center at 206-987-2515 for a referral, a second opinion or more information.

Providers, see how to refer a patient. 

Related Links

• Heart Center
• Heart Center Team
• How to Handle a Difficult Prenatal Diagnosis
• What to Expect: Heart Surgery