Single Ventricle Program

Single Ventricle Congenital Heart Disease

The Single Ventricle Program at Texas Children’s Heart Center is focused on the care of infants with single ventricle congenital heart defects, which include hypoplastic left heart syndrome (HLHS) and variants, unbalanced atrioventricular septal defects, complex single ventricle and heterotaxy syndrome, and others. Most of these infants will require at least two or three operations within the first few years of life.

The goals of these operations are to:

allow free flow of blood from the heart out to the body
protect the lungs from high pressure and too much blood flow
create separate paths for blood to flow to the lungs and out to the body

Infants will typically undergo placement of a shunt or conduit to provide blood flow to the lungs within the first few days of life. For infants with HLHS, reconstruction of the vessel that allows blood to flow to the body (the Norwood operation) is typically performed within the first week of life. The second stage (bidirectional Glenn shunt) is performed at around 3-6 months of age. The period between these operations is called interstage period.

The importance of a home monitoring program

The interstage period is an important phase in the infant’s well-being and outcome. It is common for infants with single ventricle congenital heart defects to have poor oral intake and weight gain, which may affect their well-being.

They may require multiple daily medications, modified feeding and interstage interventions. Common childhood illnesses, such as a cold or diarrhea, can be life-threatening for these children. Early recognition of signs and symptoms is thus necessary to be able to obtain immediate evaluation and intervention. The home monitoring program allows parents and caregivers to actively participate in the care of their babies outside of the hospital by watching for and addressing many of the problems that can be seen in the interstage period. Our program provides support, resources and clinical care to help families transition successfully to the second surgery.

A team approach to treatment

Texas Children’s Congenital Heart Surgery team subscribes to a collaborative approach to all patients, from fetal diagnosis through surgery and home monitoring. Comprehensive care is provided by a multidisciplinary team of experts from Cardiology, Congenital Heart Surgery, Cardiac Critical Care, Cardiovascular Anesthesiology, Genetics and Nutrition, among others.

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Research and quality improvement

We are leaders in research involving the many aspects of care of infants with single ventricle heart disease, including genetics, critical care, nutrition and epidemiology. We are part of several studies with the Pediatric Heart Network aiming to further understand and address important clinical questions that impact a patient’s care and outcomes.

Our program is also an active member of the National Pediatric Cardiology Quality Improvement Collaborative, a multicenter group focused on improving quality and outcomes for children with single ventricle heart defects who have undergone a Norwood operation.