The Single Ventricle Care Program

What is the Single Ventricle Care Program?

The Single Ventricle Care Program at Children’s Hospital Colorado offers a continuum of care from the inpatient to outpatient environment. Our multidisciplinary care approach enables families to be involved in regular communication with your child’s care team. The coordination of care from initial diagnosis to follow-up allows families to feel connected with their child’s progress.

We are committed to your child’s overall health and well-being. Our treatment plan, along with our wellness strategy, will keep you in the loop and involved in every decision.

What is single ventricle heart disease?

Some children with congenital heart defects (heart conditions present at birth) have only one effective pumping chamber. This is a condition known as single ventricle heart disease.

How is a single ventricle heart defect treated?

Single ventricle heart diseases, such as hypoplastic left heart syndrome and tricuspid atresia, are treated by performing three surgeries early in a child's life, called the Norwood, Glenn and Fontan procedures. These operations do not completely repair the heart defect, but they allow the child to grow up and a lead normal, active life. However, these children also face health challenges that are best cared for by a multidisciplinary, evidence-based program like the Single Ventricle Care Program at Children’s Colorado.

See why our outcomes make us one of the top heart hospitals

How do we help kids after cardiac surgeries?

Pediatric heart specialists in the Single Ventricle Care Program provide expert consultation and treatment of the unique issues that arise after the Norwood, Glenn and Fontan operations. For example, the Fontan circulation may affect a patient’s lungs, liver, intestines, bones, blood and other organ systems. Our specialists have expertise in evaluating and treating problems like plastic bronchitis and protein-losing enteropathy. Also, children may have everyday challenges with self-esteem and other issues in school. We have developed ways to manage those issues and others based on current research and clinical experience.

Why choose Children’s Colorado for treatment after surgery for single ventricle heart diseases

The Single Ventricle Care Program offers:

• An evaluation by a team of experts in cardiology, pulmonary medicine, liver disease and neurodevelopmental psychology. 
• Established evidence-based guidelines that encourage proactive screening for issues known to occur in children who have had a single ventricle-related operation. 
• Access to a group of experts when problems unique to single ventricle circulation occur, such as plastic bronchitis or protein-losing enteropathy.
• Support from child life specialists, child psychiatry/psychology and other providers. Families can explore concerns about their child’s quality of life, relationships with friends and family and other important non-medical aspects of life after each operation.

A lifetime of care at our Heart Institute

1. Fetal diagnosis (before birth)
2. Norwood procedure (age 0 - 1 week)
3. Glenn procedure (age 4 - 6 months)
4. Fontan procedure (age 2 - 4 years)
5. Bi-yearly visits to the Multidisciplinary Clinic (around age 2 - 4 to age 18)
6. Adult care in the Adult Congenital Heart Disease Program (age 18, for the rest of life)

The only Single Ventricle Care Program in the Rocky Mountain Region

Ours is the only program in a seven-state region dedicated to caring for all the health concerns for kids who have had a Norwood, Glenn and Fontan operation. This program is an innovative, new approach to the care of children who have had these procedures.

Access to a team of pediatric experts

Our team includes pediatric cardiologists who specialize in single ventricle heart disease. The other subspecialists on the team have a specific interest and clinical experience in single ventricle-related problems, and our multidisciplinary team allows us to provide care for all a patient’s health concerns in one place.

Our team of specialists includes:

• Pediatric cardiologists
• Pediatric gastroenterologists
• Pediatric pulmonologists
• Neurodevelopmental psychologists (specializing in treatment of developmental delays, such as missed milestones or learning difficulties)
• Child psychiatrists/psychologists (specializing in treatment of behavioral issues, like depression and anxiety)
• Other subspecialists, including physicians with expertise in the areas of clotting disorders and bone health, among others. 

Specific conditions we treat

Single ventricle heart defects are rare, only affecting a small number of children in our population. However, they are one of the most complex heart conditions, usually requiring at least one surgery.

Some of the heart conditions that we treat include:

• Hypoplastic left heart syndrome
• Tricuspid atresia (hypoplastic right heart syndrome)
• Unbalanced common atrioventricular canal
• Double-inlet left ventricle
• Double-outlet right ventricle
• All other forms of single ventricle heart disease

We also treat many issues related to single ventricle heart disease, including:

• Heart rhythm abnormalities
• Residual structural heart problems
• Decreased heart function
• Lung diseases, such as plastic bronchitis and others
• Intestinal issues, like protein-losing enteropathy and others
• Anxiety, depression and learning difficulties

Services offered

Tests:

• Echocardiogram (ECHO)
• Fetal echocardiogram
• Cardiac magnetic resonance imaging (MRI)
• Cardiac catheterization
• Electrocardiogram
• Ambulatory heart rhythm monitors (also called Holter or event monitors)
• Cardiopulmonary exercise test
• Pulmonary function testing
• Liver ultrasound
• Liver biopsy

Treatments and associated clinics include:

• Fetal Cardiology Clinic
• Colorado Fetal Care Center
• Complex Congenital Heart Disease Clinic
• Heart Transplant Program
• Adult Congenital Heart Disease Program

Single ventricle heart defect research

Our program participates in the National Pediatric Cardiology Quality Improvement Collaborative, a national research program for single ventricle heart disease. We are also an auxiliary site for the Pediatric Heart Network Fontan Udenafil Longitudinal Assessment Trial (FUEL).

Contact us

For more information or to make an appointment, please contact the Single Ventricle Care Program at singleventricle@childrenscolorado.org or by phone at 720-777-5339.