Single Ventricle Program

A single ventricle heart defect is a type of congenital heart defect (CHD) that affects the structure of a baby’s heart and how it pumps blood to the body. Typically, a baby’s heart has two ventricles, or chambers, that work together to pump blood to the lungs and to the rest of the body. Babies born with a single ventricle heart defect have only one working pumping chamber. While there are many different types of single ventricle heart conditions, some of the most common include:

• Double inlet left ventricle
• Heterotaxy
• Hypoplastic left heart syndrome
• Mitral valve atresia
• Tricuspid valve atresia or pulmonary atresia
• Unbalanced atrioventricular canal defect

Our Single Ventricle Program brings together a multidisciplinary team of specialists who are skilled in treating children with the most complex heart conditions and prioritize caring for the whole child. From diagnosis to surgery to follow-up, we offer comprehensive care tailored to each child's unique needs. We support their growth and development while promoting collaboration among the care team and family throughout this important journey.

Why Choose the Children’s Heart Center Single Ventricle Program?

• Coordinated care: Your child’s care team will consist of a cardiothoracic surgeon, a cardiologist, nurses and nurse practitioners, a social worker, speech language pathologists, physical and occupational therapists, registered dietitians, and a cardiac neurodevelopmental psychologist. Dedicated nurse practitioners will be available to your family throughout your child’s treatment to offer additional support, particularly during the interstage period (a high-risk period of time between a baby's first heart surgery and second heart surgery).
• Seamless access to your medical team: Your family will have support at every stage of your child's treatment. We will be available to answer your questions, provide guidance and monitor for signs of complications. You can expect close surveillance by your care team, frequent outpatient clinic visits, weekly phone calls, routine testing and 24-hour availability by phone.
• Excellent outcomes: At the Children’s Heart Center, our cardiothoracic surgeons perform more heart surgeries than anyone else in Georgia*, with outcomes that exceed the national average. You can feel confident your child is being cared for by an experienced team of heart specialists.

The Children’s Heart Center Single Ventricle Program is part of the Single Ventricle Outcomes Network, which is a collaborative community of some of the nation’s leading providers of pediatric heart care. Together, we are working to share knowledge and accelerate discoveries that will help improve the care and outcomes of children with single ventricle heart conditions.

Single ventricle heart conditions are typically first noticed during pregnancy by an obstetrical or prenatal ultrasound. If your doctor suspects that your baby has a single ventricle heart condition, you will be referred to a fetal cardiologist who will perform a fetal echocardiogram to look at your baby’s heart and its structures to confirm the diagnosis.

Pediatric heart surgery

Children with single ventricle conditions typically require multiple surgeries, also known as staged reconstruction, to re-route blood flow and help the heart function more effectively. While there are different types of single ventricle heart conditions, treatment typically involves three surgeries that are performed at different times throughout childhood.

• Norwood procedure: The Norwood procedure is usually performed within the first week of an infant’s life. During this procedure, the surgeon creates a new, larger aorta by combining the original tiny aorta with a healthy pulmonary artery, which usually pumps blood to the lungs. This allows blood to flow freely to the body. Then, a small tube, also known as a shunt, is placed either from an artery to the pulmonary arteries (called a modified Blalock-Taussig-Thomas shunt) or from the right ventricle to the pulmonary arteries (called a Sano shunt).
• Bidirectional Glenn procedure: Typically performed when a child is 4 to 6 months old, this surgical procedure involves connecting a large vein, called the superior vena cava, to the pulmonary arteries, which allows blood that needs oxygen to go directly to the lungs. By bypassing blood away from the small ventricle, this surgery can take some stress off the right ventricle for a few years until the next heart surgery is needed.
• Fontan procedure: This procedure is usually performed when a child is 3 to 5 years old. During heart surgery, the large vein that returns blood from the lower part of the body to the heart, the inferior vena cava, is connected to the pulmonary artery, which allows blood coming back from the body to go directly to the lungs. Once this is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart, and the child’s blood oxygen levels become normal.

The interstage period is a high-risk period of time between a baby’s first heart surgery and second surgery. Studies show that home monitoring helps improve outcomes during this period, and we strive to educate and prepare you to care for your child at home.

In addition to outpatient clinic visits, weekly phone calls, routine testing and 24-hour phone access, your team will work closely with you to set up a home monitoring plan, so that you can actively participate in your child’s care outside of the hospital and help ease their transition to the second surgery. When your baby is discharged, you will receive an infant scale and pulse oximeter. You will need to carefully track your baby’s oxygen levels, weight and fluid intake and enter data into the patient portal daily. The single ventricle team will assess your child’s progress daily, looking for any potential problems, and will contact your family at least once a week.

Tips for successful home monitoring after hospital discharge

• Communicate with your child's medical team.
• Let us know about any concerns, questions, as well as all accomplishments.
• Don't be afraid to ask for help.
• We know that it can be scary and challenging to go home with a fragile baby. We encourage you to rely on us to provide help and resources during this time.
• Keep a calendar or journal.
• A calendar will help you keep track of important details and dates regarding your child's treatment, such as medications, feedings, home monitoring and appointments.

Continuing Care

If your child was born with a single ventricle heart condition, they will need to see a pediatric cardiologist throughout their childhood. At the Children’s Heart Center, we focus not only on promoting good heart health, but growth and development from infancy through adulthood. Your child may be required to take heart medications that help the heart work as efficiently as possible and will undergo frequent testing to monitor their heart health. In some cases, a heart transplant may be needed if their heart becomes weak over time.

Cardiothoracic surgeons

• Subhadra Shashidharan, MD, Surgical Director
• Paul Chai, MD, Co-Chief, Heart Center; Chief, Cardiothoracic Surgery

Cardiologists

• Timothy Slesnick, MD, Medical Director
• James Nelson, MD
• Alyson Pierick, MD
• Marcos Mills, MD
• James Kuo, MD

Advanced practice providers 

• Megan Baker, CPNP-AC
• Nicole Coolidge, CPNP-AC, FNP-C
• Shelby Corder, CPNP-AC

Single ventricle surgeries are performed at Arthur M. Blank Hospital.

Arthur M. Blank Hospital
2220 North Druid Hills Road NE
Atlanta, GA 30329