Single Ventricle Clinic

Having a child born with a single ventricle heart defect such as hypoplastic left heart syndrome can be very scary. That’s why our team at the Single Ventricle Clinic is here to provide comprehensive multidisciplinary care for your child from birth through the transition to the adult congenital heart program.

What is a single ventricle heart defect?

Babies born with single ventricle heart disease only have one working pumping chamber (ventricle) instead of two. This condition is often diagnosed during pregnancy with a fetal echocardiogram or immediately after birth with an echocardiogram.

Single ventricle heart defects — some of the most challenging and serious heart conditions — require a series of open heart surgeries early in life called the Norwood, Glenn and Fontan procedures. During these procedures, our pediatric cardiothoracic surgeons reconfigure the heart and reroute blood vessels to provide stable blood flow and oxygen delivery to the body.

Who is cared for at the clinic?

Our experts at the Single Ventricle Clinic provide comprehensive care to children born with single ventricle heart defects. These children need specialized, lifelong follow-up care.

For babies diagnosed with a single ventricle heart defect before birth, we’re here to meet with you and answer any questions during your visit with our fetal cardiology team.

After your child’s Norwood procedure, which takes place shortly after birth, we’ll see them during the high risk interstage period at the Advocate Children’s Hospital in Oak Lawn.

The Glenn procedure takes place around 4-6 months of age, and helps improve oxygen flow. After recovery from the procedure, clinic visits can continue at our Oak Lawn location or be transitioned to the Advocate Children’s Hospital in Park Ridge.

Once your child is around 2 years old, we'll perform the last surgery, the Fontan procedure. During this procedure, blood flow from the lower part of the body is directed to both lungs, effectively bypassing the pumping heart chamber that didn't form during fetal life. Although most children go on to lead active lives after the Fontan procedure, they may face ongoing health challenges that are best cared for by a multidisciplinary team. Our team provides continuity of care for these patients until transition to our adult congenital heart disease team in adolescence.

How is my child cared for in the home monitoring program?

During the interstage period between the first and second heart surgeries, it’s common for infants with single ventricle heart defects to have poor feeding, poor weight gain and other potential problems which may affect their well-being.

Once your child is ready for discharge after the Norwood procedure, our interstage home monitoring team will provide you support from a group of experts in cardiology, nutrition, social work, feeding therapy and neurodevelopment. This support includes learning how to:

• Use an oxygen monitor device to check your child’s heart rates and oxygen levels at home
• Give medications
• Obtain daily weights
• Use a feeding tube to provide optimal nutrition, if needed

We’ll make sure you’re comfortable doing all of this prior to your child’s discharge from the hospital. You’ll also receive:

• Take-home education
• Weekly phone calls from a dedicated nurse practitioner as a familiar face throughout the interstage period
• Support from a dietician and team of therapists including speech pathology, physical therapy and occupational therapy
• Pager for direct access to a single ventricle cardiologist 24/7

Tube weaning program

After their first heart surgery (the Norwood procedure), most children are reliant on a feeding tube at the time of discharge for optimal nutrition. Our team of advance practice nurses, dieticians and therapists are available at every clinic visit to assess your child's continuing progress in building strength and coordination to take feeds by the mouth.

After recovery from the second heart surgery (the Glenn procedure), we’re here to help if your child is still on a feeding tube. We have a tube weaning program to help your child come off the feeding tube, and advance to eating all by mouth as quickly and as safely as possible.

Monitoring and supporting neurodevelopment

Research shows that children with single ventricle heart defects are at risk for a variety of long term neurodevelopmental and psychosocial issues, even if they seem to be meeting their developmental milestones early on.

That’s why your child will be referred to our cardiac neurodevelopmental clinic for full developmental evaluation every six months until age 3. They’ll be seen again at age 5 for school readiness evaluation and assistance with a 504 plan or individualized education program as necessary.

The transition from childhood to adulthood

As your child ages and becomes a young adult, we’ll be there to help with the transition of care to our excellent adult congenital heart disease team, who is trained to care for adults with single ventricle heart disease.

Conditions we treat

We have the expertise to treat a variety of different heart defects including:

• Complex single ventricle
• Hypoplastic left heart syndrome and variants
• Hypoplastic right heart syndrome
• Interrupted aortic arch
• Aortic atresia
• Pulmonary atresia
• Tricuspid atresia
• Double inlet left ventricle
• Double outlet right ventricle
• Unbalanced atrioventricular septal defect
• Ebstein’s anomaly
• Heterotaxy (isomerism) syndromes

Meet the single ventricle heart defects team.