Single Ventricle Care Program

Families who receive a prenatal diagnosis of complex fetal heart disease receive care at the MUSC Children's Health Advanced Fetal Care Center (AFCC). The AFCC team includes fetal cardiologists, maternal-fetal medicine specialists, family support providers, genetic counselors, and nurse care coordinators. The team at AFCC provides education and information about the fetal cardiac diagnosis, delivery plan, what to expect after birth, and overall care and prognosis for a child living with a complex heart defect. The Single Ventricle team works closely with the AFCC team for all families who receive a prenatal diagnosis of single ventricle heart disease.

Most babies with a single ventricle heart defect need surgery or intervention within the first 1-2 weeks after birth. The Single Ventricle team works closely with the cardiac intensive care unit (CICU) and cardiac step-down unit (CSD). When the baby is close to leaving the hospital, the Single Ventricle team helps prepare the family for the transition from the hospital to home. This preparation includes family education and training, developing medical emergency plans, coordinating appointments and therapies, and communicating with the local medical teams close to the baby’s home.

The “interstage period” refers to the time between the first and second surgeries for infants living with a single ventricle heart defect. During the interstage period, babies are fragile and at high risk for poor growth, sickness, breathing problems, and sudden unexpected death. The Interstage Monitoring Program is led by the Single Ventricle team and helps monitor and care for babies and provide family support during the interstage period. The Interstage Monitoring Program includes pediatric cardiologists, nurse practitioners, dietitians, and a home health team called Hands of Hope. The Interstage Monitoring Program provides specialized care including education and training for families, and infant scale and pulse oximeter for daily home use, weekly phone calls to families, and weekly communication to the local cardiologist and pediatrician. During the interstage period, infants will be evaluated in the Cardiac Neurodevelopment Clinic by a pediatric cardiologist, therapists (speech, occupational, and physical), and a dietitian.

For most babies with a single ventricle heart defect, the second stage heart surgery is the bidirectional Glenn procedure. After the bidirectional Glenn, babies are usually stronger and able to “graduate” from the Interstage Monitoring Program. Infants continue to be followed closely by their local cardiologist. After the bidirectional Glenn, the Single Ventricle team will still be involved with the care and will help coordinate specialized cardiac testing, weaning from the feeding tube, and follow-up with pediatric neurodevelopment specialists. After the bidirectional Glenn procedure, the Single Ventricle team will evaluate infants and children every 6 to 12 months until the Fontan procedure.

Most children with a single ventricle heart defect have the Fontan procedure between 3-5 years of age. After the Fontan procedure, children continue to be followed by their local cardiologist as well as the Single Ventricle team. One year after the Fontan procedure, children are seen in the Fontan Junior Clinic for detailed cardiac testing, neurodevelopment evaluation, and nutrition monitoring. Five years after their Fontan procedure, children are transitioned to the Longitudinal Fontan Care Clinic where they receive yearly evaluations by a highly specialized team. This team monitors the heart and other organs that are affected by the Fontan circulation and determines if a heart transplant evaluation is necessary.

Having a child with a single ventricle heart defect can be stressful and overwhelming. The Single Ventricle team partners with a home health organization called Hands of Hope that provides in-home nursing visits during the Interstage period and provides family support throughout the entire single ventricle journey. During hospitalizations, patients and families are supported by a team that includes a pediatric psychologist, a social worker, a chaplain, and the palliative care team. We also connect families with community support organizations like the Emerson Rose Heart Foundation, the HEARTest Yard, and the Children’s Heart Foundation who help families navigate this journey and provide support along the way.

Patients are evaluated by a pediatric cardiologist, a developmental pediatrician, a registered dietitian, and multiple therapists (physical, occupational, speech). This clinic ensures children are growing well mentally and physically.

Patients are evaluated by a pediatric cardiologist who specializes in the Fontan circulation, a member of the developmental pediatric team, and a registered dietitian. Patients are typically seen a year after their Fontan procedure and followed intermittently until transitioned to the Longitudinal Fontan Care Clinic.

Patients are evaluated by Fontan cardiologists, a hepatologist (liver specialist), the Pediatric Advanced Cardiac Therapies (PACT) team, a nephrologist (kidney specialist), the family support team, and a dietitian. Prior to the clinic appointment, important testing such as blood work, exercise stress tests, and imaging of the heart and liver are coordinated to prepare for the clinic appointment so the Fontan team can develop a comprehensive care plan.