Pediatric Single Ventricle

Pediatric Care for Single Ventricle Heart Defects

Specialists at University Hospitals Rainbow Babies & Children’s Hospital are dedicated to helping children and families cope with the challenges of a single ventricle diagnosis.

We are widely recognized for our leadership in staged surgeries to help relieve patients’ symptoms and improve quality of life for single or dominant ventricle heart issues, such as for hypoplastic left heart syndrome, tricuspid atresia and many others.

What is Single Ventricle Defect?

Approximately 1 in 10,000 children are born with a single ventricle heart defect. It occurs when one of the two pumping chambers in the heart, called ventricles, isn’t large enough or strong enough to work correctly or is missing a valve. One of the most serious forms of congenital heart disease, a single ventricle defect prevents the heart from pumping blood effectively.

This heart condition is usually diagnosed before birth via a fetal echocardiogram, a special type of ultrasound to look at the baby’s developing heart. In most cases, it requires intensive medical and surgical intervention soon after delivery. To repair the defect, children typically will undergo a series of staged surgeries during the first three years of life.

Numerous cardiac conditions are considered single ventricle defects, including:

Benefits of Single Ventricle Monitoring Program

The Single Ventricle Monitoring Program provided through the Congenital Heart Collaborative at UH Rainbow Babies & Children’s Hospital offers specialized care for high-risk infants born with a single ventricle heart defect. These infants are monitored very closely during all the stages between their necessary heart operations. The goal of the program is to prevent life-threatening events, maximize growth and development and educate and support families.

Families whose child is in the monitoring program benefit from:

  • Comprehensive management from the medical and surgical teams handling the most complex cases – from prenatal diagnosis through treatment and lifelong follow-up care.
  • Structured educational resources and support to reduce stress and anxiety for parents and improve quality of life for their child.
  • Weekly multidisciplinary clinics that bring together the pediatric cardiologist, nurse practitioner, cardiac nursing specialists, nutritionist, social services, feeding therapist, child life therapists and neurodevelopment experts to support your child's overall health between operations.
  • A dedicated program that focuses on the care of infants between heart surgeries, whether they are being cared for in the hospital or at home.
  • Our team's participation in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a national project dedicated to improving survival and quality of life for infants with single ventricle heart defects, not only during the inter-stage periods but now through the first year of life.
  • We are the first pediatric heart program in northern Ohio to provide a home monitoring system, called Hearts at Home. This advanced technology helps give our parents more confidence in caring for their infants between surgeries to treat single ventricle defect. In addition to monitoring, this system also provides access to the pediatric cardiology team, 24/7.

Hearts at Home monitoring

Improved Quality of Life with a Single Ventricle Defect

Children who undergo surgical repair for single ventricle defects require lifelong follow-up care by a pediatric cardiologist and may need to take certain medications for the rest of their life. Sometimes additional procedures will be required throughout the patient’s lifetime.

You can promote your child’s health and minimize their risk of complications by encouraging them to:

  • Avoid smoking
  • Eat a healthy diet consisting of lots of fruits, vegetables and whole grains, but low in fats, sweets and salt
  • Maintain a “normal” BMI
  • Maintain a normal blood pressure
  • Maintain excellent oral health
  • Maintain regular clinic follow-up
  • Participate in some form of exercise as prescribed by the cardiologist or nurse practitioner
  • Take their medications as prescribed

For More Information about Single Ventricle Care

If you or a family member has any questions regarding our Single Ventricle Monitoring Program, please call 216-844-1234 to speak with a team member.

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