Single Ventricle Comprehensive Program

The Single Ventricle Comprehensive Program provides complete, lifelong care for children who have undergone the surgeries to correct a single ventricle heart defect. The program allows a patient's primary cardiologist to work closely with specialists in this congenital heart condition, ensuring that each child receives the best possible care.

A single ventricle defect is when only one of the heart's pumping chambers works well enough to effectively circulate blood to the lungs and rest of the body. Treatment involves a series of three corrective surgeries that ultimately allows deoxygenated blood – blood that has delivered oxygen to body cells – to flow passively into the lungs (without being pumped by the heart) while routing the oxygenated blood to the single working ventricle (to be pumped to the body).

At UCSF, children with single ventricle defects have access to outstanding care, including our state-of-the-art fetal cardiovascular program, a carefully integrated cardiothoracic surgery program and our pediatric cardiac intensive care units. Following the first corrective surgery (Norwood procedure), our rigorous interstage home monitoring program provides families with the tools and resources to successfully prepare their babies for the second surgery (Glenn procedure). After patients undergo their third and final surgery (Fontan procedure), the Single Ventricle Comprehensive Program provides consultative services for the long-term care they may need.

Expert ongoing care is crucial because patients with single ventricle anatomy are at a higher risk for a wide variety of conditions, including problems with the heart, lungs, liver and kidneys, as well as issues with growth, learning and psychosocial development. In an effort to catch problems early, we periodically conduct comprehensive evaluations that include age-specific screening tests. We may order blood, urine or stool tests; imaging studies, such as ultrasound or MRI; cardiac catheterization; exercise tests; and neurodevelopmental evaluations.

All test results are reviewed by our team of specialists. Working together, they summarize the findings and provide recommendations to the family and the child's primary cardiologist. For each patient, we conduct two or three evaluations in the period between receiving the Fontan procedure and reaching adulthood. As patients approach maturity, we facilitate a seamless transition to adult care by including our adult congenital heart disease team in these discussions. Our shared goal is to ensure long-term quality of life for patients with single ventricle physiology.