Children born with single ventricle heart defects need an experienced team of pediatric heart surgeons, cardiologists, and other experts to provide trustworthy, quality care from before birth through adulthood. While rare, single ventricle heart defects are complex problems that often require a series of surgeries. The mission of Duke’s single ventricle program, the first of its kind in North Carolina, is to improve survival and quality of life for children born with single ventricle heart disease. 

Our care goes beyond surgery to include:

  • At-home monitoring for kids who are between major surgeries
  • Neurodevelopmental care to support learning and development
  • Specialty follow-up care for children who have undergone Fontan surgery
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Types of Single Ventricle Heart Defects We Treat

We treat many types of single ventricle defects, including:

  • Hypoplastic left heart syndrome
  • Unbalanced complete atrioventricular (AV) canal defect
  • Double inlet left ventricle
  • Double outlet right ventricle
  • Pulmonary atresia with intact ventricular septum
  • Complex transposition of the great arteries 
  • Tricuspid atresia

How We Treat Single Ventricle Heart Defects

Treating single ventricle defects requires highly specialized, coordinated care for life that starts before birth. Duke’s team of experts includes pediatric and adult heart surgeons, cardiologists, interventional cardiologists, critical and intensive care experts, anesthesiologists, and a host of other specialists who work hand-in-hand to ensure your child receives the very best care. Our providers meet regularly in a single ventricle program conference to discuss and customize treatment plans for each child in our care. We offer individualized care and follow-up regularly after discharge through our interstage monitoring program.

Single ventricle heart defects are usually discovered during a routine second-trimester ultrasound. Our fetal cardiology experts may order more advanced imaging. This could include targeted fetal ultrasound, fetal echocardiography (ultrasound that studies your baby’s heart and blood flow), and MRI.

Preparing for Your Child’s Birth
Before your baby is born, we meet with you to discuss what you can expect. We’ll talk about plans for your baby’s delivery at Duke Birthing Center at Duke University Hospital and answer any questions you have. To help you feel more comfortable, you can tour our facilities, including our specialized Pediatric Cardiac Intensive Care Unit and our Level IV Neonatal Intensive Care Unit (the Level IV designation indicates we provide the highest standard of care for critically ill infants). After your child is born, they will be cared for around-the-clock by pediatric and neonatal specialists -- including pharmacologists, respiratory therapists, and nutritionists -- with expertise in caring for newborns who have complex medical conditions.

Heart Surgery
A child born with a single ventricle defect will need one or more surgeries in the first few years of life. For example, these may include the Norwood, Glenn, and Fontan operations (together, these are called “single ventricle palliation” and they re-plumb the heart to work with just one functioning ventricle). Other surgeries are common as well. Our pediatric heart surgeons regularly perform (and in some cases have pioneered) these incredibly complex and otherwise rare heart surgeries.

Advanced Surgeries to Restore Normal Heart Circulation

Your child may be eligible for new surgical approaches that help the heart function more normally. These could replace one or more of the traditional heart surgeries (single ventricle palliation) that your child would otherwise need in their lifetime.

  • Ventricular Recruitment: Through a series of procedures that direct blood flow, surgeons engage the smaller ventricle and encourage it to grow. This allows for normal, biventricular repair later on.
  • Biventricular Repair: Surgically rearranges the heart’s connections to allow the heart to function more normally.
  • Biventricular Conversion: This procedure is for children who have already had surgeries to compensate for the smaller or non-functioning ventricle. It converts the heart back to what’s called double-ventricle circulation (blood can flow through two ventricles, instead of just one).

Exhaustive Care Beyond Surgery

At-Home Monitoring Between Surgeries
Children can be especially vulnerable between their heart surgeries, known as the interstage period. We provide equipment and teach you how to monitor vital measures like blood pressure, weight, and oxygen saturation at home. We show you how to identify warning signs, and we provide a way for you to contact us directly in case of emergency.
 Using telehealth visits, a dedicated nurse practitioner will check in with you often and help coordinate care between your child's Duke doctors and local providers.

Neurodevelopmental Care
Because heart defects can affect how a child develops and learns, our Pediatric Cardiac Neurodevelopmental Clinic is staffed by pediatricians, occupational therapists, physical therapists, speech-language therapists, neuropsychologists, dietitians, social workers, and nurses who are dedicated to monitoring mental development, nutrition, growth, and other factors in high-risk infants with complex congenital heart disease.

Post-Fontan Care
People who have had a Fontan operation (the final surgery in the series that makes up single ventricle palliation) are at an increased risk of complications and other health problems. Duke’s variety of specialists want to check on your post-Fontan health regularly -- from nutrition to cardiology, hepatology, endocrinology, physical therapy, and beyond.

Our Locations

Pediatric heart surgery is performed at Duke University Hospital. Pre- and post-surgical appointments may take place at Duke Children's Health Center or at other clinics in the region.

Why Choose Duke

Highly Experienced with Great Outcomes
Research shows that hospitals and surgeons who perform more surgeries tend to have better outcomes. Although single ventricle defects are relatively rare, these conditions account for nearly a quarter of all the heart surgeries we perform. 

Recognized for Excellence
Duke Children’s Hospital is verified as a Level I Children's Surgery Center by the American College of Surgeons. This Level I designation recognizes our commitment to providing the safest and highest-quality surgical care to our young patients. 

Specialized Intensive Care
Babies with congenital defects are transitioned immediately to one of our specialized intensive care units and cared for by neonatologists and other pediatric specialists. Our Pediatric Cardiac Intensive Care Unit (PCICU) is staffed by physicians with specific training in critical and heart care. Our Neonatal Intensive Care Unit (NICU) has a Level IV designation, indicating we provide the highest level of care for critically ill infants. Once babies have stabilized from their more acute conditions, they move to nearby step-down units. There, they continue to grow and develop skills -- such as feeding -- that are needed before they can safely transition home.

A Personal Touch with Professional Expertise
Our single ventricle program’s dedicated nurse practitioner builds personal connections with each child in our care and their families. Their involvement extends beyond surgery, post-op, and even discharge through frequent check-ins and virtual monitoring via an app developed by Duke providers and parents. We help coordinate care, educate, and communication between their local pediatricians and cardiologist to ensure they are all working as a team to provide the best care for your child.

Family Participation
We go above and beyond to incorporate as many of your preferences as possible. Parents are treated as part of their child’s care team. Our lactation consultants support new mothers to provide their own milk for their babies while hospitalized; and we can provide donor human milk from an HMBANA-certified milk bank when a mother’s milk is not available. We also encourage kangaroo care -- this skin-to-skin contact has proven benefits, including stabilizing your baby’s heart rate and helping to regulate body temperature.

Support Services
Our family-centered care includes support groups, wellness programs, and education for siblings to help them understand what is happening to their brother or sister. We have a Ronald McDonald House Family Room that offers amenities to make your family’s stay as comfortable as possible.

Improving Care
We’re always looking for ways to advance single ventricle heart disease treatment. In addition to clinical trials that could get you access to new therapies faster, we also maintain internal databases that we use for patient monitoring, comparative effectiveness research, and quality improvement. Also, Duke is one of 69 centers in the United States and Canada that participate in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), which aims to improve survival and quality of life in infants with certain single ventricle heart defects. 

Quality of Life
Our Neonatal Quality of Life Program is for newborns and infants with life-threatening, and potentially life-limiting, conditions. A team of doctors, nurse practitioners, spiritual counselors, and social workers specialize in maximizing comfort and providing pain and symptom relief. They also provide emotional, psychosocial, and spiritual support for your family. Our Child Life program helps your young child cope with hospitalization through play, procedure preparation, and self-expression activities. They can meet other children going through similar experiences in our activity room.

A gold badge shows Duke has been nationally ranked in 10 pediatric specialties for 2023 to 2024
#2 in Nation and #1 in NC for Pediatric Cardiology and Heart Surgery

Duke Children’s is ranked the #2 pediatric cardiology program in the nation and the best in North Carolina by U.S. News & World Report.

This page was medically reviewed on 10/14/2022 by