Single Ventricle Clinic

Single ventricle heart conditions, such as hypoplastic left heart syndrome and tricuspid atresia, are treated through a series of two or three surgeries in the first few years of a child’s life, including the Fontan procedure. 

Thanks to advances in surgical techniques and post-surgical care, survival rates for patients with single ventricle heart conditions continue to improve.

While surgery to establish normal blood circulation can be effective, specialized long-term follow-up care is essential to ensure patients have every opportunity to lead normal, active lives.

The multidisciplinary Single Ventricle Clinic provides comprehensive longitudinal evaluation and follow-up care for children, adolescents and young adults with single ventricle congenital heart disorders.

Health concerns for single ventricle patients

While many single ventricle patients lead full-active lives, they are at higher risk for a number of issues, including:

  • Fatigue and difficulty exercising
  • Concerns with growth and development
  • Abnormal heart rhythms
  • Respiratory and sleep issues
  • Liver and intestinal disorders
  • Bone health and growth abnormalities
  • Problems with blood and other organ systems
  • Plastic bronchitis (PB) and protein-losing enteropathy (PLE)
  • Social adjustment
  • School performance
  • Anxiety & Depression
  • Kidney disease

Proactive screening for these conditions represents the most effective way to detect and manage issues before they lead to more serious health problems. 

Our team of specialists uses an evidence-based approach to identify and manage risk for long-term health issues based on current research and our extensive clinical experience.

Group photo of the single ventricle team

Multidisciplinary team approach

As the health issues that single ventricle patients experience can involve multiple organ systems, the multidisciplinary Single Ventricle Clinic uses a team approach to provide collaborative evaluation and treatment.

Our program brings together pediatric specialists in:

  • Cardiology - who specialize in diagnosing and treating individuals with congenital heart disease, and specifically in this clinic, focused on individuals with Fontan physiology
  • Gastroenterology and Hepatology – who specialize in diagnosis and management of patients with all types of liver disease
  • Nephrology – who specialize in the treatment of disorders of the kidneys, urinary tract and high blood pressure
  • Pulmonology –who specialize in diagnosis and treatment of breathing disorders and lung disease
  • Genetics – who specialize in diagnosing and treating disorders and congenital abnormalities
  • Psychology – who specialize in providing comprehensive treatment of developmental, behavioral, growth and illness related issues affecting children, adolescents and their families 

Who we can help

It is recommended that all single ventricle patients receive a comprehensive evaluation every 3-5 years to consider the effects of Fontan circulation on the whole body.  For your convenience, consultations and any necessary exams will be coordinated to allow for patients to be seen by all appropriate specialists within 1-2 days.  If appropriate, patients may also be scheduled for evaluation in our neurodevelopmental follow-up clinic.

Evaluation by the multidisciplinary Single Ventricle Clinic team is meant to be in addition to routine care provided by your child’s primary cardiologist.  Referring physicians will receive a comprehensive summary of our team’s findings and follow-up recommendations.

What testing will be performed during your visit 

  • Echocardiogram – a detailed ultrasound of the heart to look at the structures and heart function
  • Electrocardiogram – a fast and easy test to check the electrical activity of the heart that includes placing stickers on your chest, arms and legs
  • Exercise stress test - this test provides information on how a heart functions and responds to exercise.  In this clinic this testing is most often completed on a stationary bicycle. The test will get progressively harder over time.  Blood pressure, heart rate, heart rhythm and oxygen saturations will be measured before, during and after the test
  • Cardiac MRI - a test using a powerful magnet, radio waves and a computer to take clear pictures of the heart.  An MRI exam is painless. You won’t feel the magnetic field or radio waves. MRI machines consist of a large magnet shaped like a tunnel. Your child lies on a table that slides into the tunnel. A computer creates a composite of 2 and 3-dimensional representation of your body
  • Liver ultrasound - an ultrasound of the liver and blood vessels entering and exiting the liver
  • Kidney ultrasound - an ultrasound of the kidney, ureters and bladder
  • Pulmonary Function testing - measures how well you can move air in and out of your lungs 

How can I prepare for my appointment? 

Your child’s testing and visit in the single ventricle clinic will take most of the day. Your child will need to be fasting for the liver ultrasound, so please bring snacks and a drink to have after the ultrasound is complete. Make sure to wear comfortable clothes and tennis shoes for the exercise stress testing. 

Research to improve long-term health for single ventricle patients

As one of the nation’s leading children’s heart centers, our faculty are involved in groundbreaking research studies to advance understanding of the long-term health and quality-of-life for people with single ventricle circulation. There may be opportunities for you and your child to participate in research. Current research topics include:

  • Increasing exercise tolerance  
  • Advancing mental health care 
  • Improving neurodevelopmental outcomes  
  • Promoting health care equity 
  • Managing and reducing complications, such as protein losing enteropathy, plastic bronchitis and Fontan-associated liver disease 

These studies focus on long-term survival and quality of life in patients with single ventricle circulation. We are committed to increasing the heart community’s collective knowledge about single ventricle diseases and how they affect patients throughout every phase of life. 

Make an appointment

To make an appointment or learn more about our multidisciplinary Single Ventricle Clinic, call 734-764-5176 and ask to speak with Suzanne Viers about this program.

Go back to the Congenital Heart Overview Programs and Clinics page.