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Single Ventricle Follow-up Clinic

After the Fontan operation at 3-4 years of age, children with single ventricle physiology can be followed in the Single Ventricle Follow-Up Clinic by the Single Ventricle Center of Excellence team at the main hospital. Patients with single ventricle physiology are usually seen once or twice a year, depending on the child’s cardiac function. 

The Team

The Single Ventricle Clinic team is made up of a nurse coordinator, two advanced practice providers, and four dedicated cardiologists. Children will typically be seen by the same providers; however, all our providers know all of our patients. Prior to every appointment, our entire team reviews each patient and their care plan to ensure the best possible care is provided. This specialized team partners with: 

  • Electrophysiology – Patients born with single ventricle physiology are at high risk for developing problems with their heart rhythmTheir heart could either beat too fast or too slow due to missing or faulty anatomy or injury caused during a procedure. Testing such as an EKG24-hour Holter monitor, 30-day event monitor, or stress test can be done to diagnose a rhythm issue. The Electrophysiology team may prescribe medications or recommend a pacemaker to regulate your heart rate.  
  • Adult Congenital Heart Disease – Patients born with a congenital heart defect (CHD) require lifelong, specialized cardiac care and this program’s cardiologists have specific training and experience in caring for adolescents and adults with CHD. 
  • Nutrition – Good nutrition positively impacts surgical and neurodevelopmental outcomes making it very important for patients with single ventricle heart defects. A registered dietician can determine the number of calories each patient needs and formulate an individualized nutrition plan.
  • Hepatology – Due to increasing evidence that liver disease is a common complication of Fontan physiology, the Single Ventricle Clinic team increased the frequency of liver screening including regular blood work, liver ultrasound, and liver biopsy. Here at Lurie Children’s, patients will be screened with blood work and imaging on a regular basis to monitor for concerning changes in liver function. 
  • Exercise Physiology - Exercise is important for all patients, especially those with CHD. While we want all our patients to exercise, we want to make sure they are doing it in the safest way possible. 

While they are not embedded in the clinic, the Single Ventricle of Excellence Team also works closely with specialists in all of the other major pediatric subspecialties that may affect our patients – commonly neurology, psychology, endocrinology, immunologyhematology, nephrology, cardiovascular genetics, and infectious diseases

What to Expect

At each visit to the Single Ventricle Clinic, patients will typically have cardiac testing (Echocardiogram and EKG) and have a visit with our Single Ventricle Clinic Team. Any of the following testing could be added to a clinic appointment: 

  • 24hr Holter monitor (heart rhythm monitor) 
  • Lab/blood work 
  • Nutrition counseling 
  • Liver Ultrasound with elastography* 
  • 6 Minute Walk Test and exercise counseling 
  • Stress Test 

*Starting four years post Fontan surgery, patients will start with annual liver ultrasound with elastography and consultation with the Liver specialist. 

Neurodevelopment

Research shows that children with single ventricle physiology are at risk for a variety of neurodevelopmental and psychosocial issues in the long term, even if they seem to be meeting their developmental milestones early on. Continued and careful surveillance by a team with specific expertise in the challenges faced by our cardiac patients is critical to ensure these children have the best possible long term functional outcome and quality of life. With this in mind, we refer many patients to the NICU-Cardiac Neurodevelopmental Program (NCNP) at Lurie Children’s. This is one of only a few programs in the country that provides individualized care to ensure these high-risk children reach their full potential later in life. The multidisciplinary team of experts in the NCNP program works with children and their families to identify and address any developmental concerns and to help families build a solid foundation for later learning and future success. The program follows children from birth through graduation from high school.  

See a list of developmental milestones