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Single Ventricle Care Program

Heart illustration showing fontan circulation

Children with single ventricle heart defects undergo a series of staged surgeries during the first few years of life. These operations result in a reconstructed circulation called the Fontan circulation. This unique circulation has allowed made it possible for children with single ventricle heart disease to grow and thrive into adulthood, however they require highly specialized care to address the many unique outcomes of their circulation.

The Single Ventricle Care Program is committed to providing lifelong multidisciplinary care and support for children and adults living with single ventricle heart defects. This care program includes teamwork with medical specialists in all areas affected by this unique circulation including dietician support, neuropsychiatry, feeding therapy, kidney providers, liver specialists, family support, palliative care and symptom management, advanced cardiac therapies, and care coordination with your primary cardiologist.

The period between the newborn operation (Norwood, hybrid or shunt) and second stage operation (Hemi-Fontan or bidirectional Glenn) is known as the interstage period. The interstage period is an especially vulnerable time for single ventricle babies and requires specialized care and monitoring.

Interstage Monitoring Program

The mission of the Interstage Monitoring Program is to prevent life-threatening events and improve growth and quality of life between the first hospitalization and second-stage surgery, known as the “interstage” period. The core interstage monitoring team includes cardiologists, a nurse practitioner and a dietitian.

Services Offered by the Interstage Monitoring Program

  • Continuum of care from the inpatient to outpatient environment
  • Ongoing and comprehensive education and training from a specialized nurse practitioner
  • Care coordination for the transition from the hospital to home
  • Written education resources including an personalizedpatient care binder
  • Home monitoring with an infant scale, pulse oximeter and mobile health technology
  • Family support services in the home environment
  • Weekly phone communication with the program nurse practitioner
  • Coordination of care and services including follow-up feeding evaluations, cardiac catheterizations and other sub-specialty appointments

The Fontan Longitudinal Care Clinic

Children who have had the Fontan operation are living longer now. However, there are unique medical issues that occur which need specialized health care. The Fontan Longitudinal Care Clinic at MUSC Children’s Health is one of a few national centers, and the only dedicated center in South Carolina, that provides this focused care to patients who have had the Fontan operation.

The Fontan clinic is comprised of a large team of pediatric specialists familiar with the unique issues in Fontan circulation. This includes pediatric and adult congenital cardiology, heart-failure specialists ("Pediatric Advanced Cardiac Therapies"), heart rhythm specialists (electrophysiology and pacemaker technician), liver specialists (hepatology), kidney specialists (nephrology), and a family support team. A social worker is also present for questions and family assistance. In addition, our team has direct referral pathways to other sub-specialties including endocrinology, pulmonology, neuropsychology, obstetrics and gynecology, dieticians, and more.

All of the providers have expertise in how the Fontan circulation affects many organ systems, and the team collaborates to develop recommendations for each patient. A detailed assessment with all the MUSC Children's Health specialists' recommendations is communicated to the patient's primary cardiologist.

Goals of the Fontan Longitudinal Care Clinic

  • Supplement care currently being provided by the patient’s cardiologist. All recommendations are communicated back to your primary cardiologist
  • Provide complete care based on national guidelines for children and adults with Fontan circulation
  • Organize the many recommended tests and sub-specialty visits into a single visit for patient and family convenience
  • Allow patients and families to hear their personal results from each Fontan team provider and ask questions to learn more about their health

The evaluation process is normally conducted over two separate days. The first day is for Fontan testing and the second day is the clinic visit to discuss the test results with the Fontan team. These days are usually two to three weeks apart to give the team time to interpret the test results and create a care plan. Our single ventricle coordinator organizes the testing and clinic visits for your family. The Fontan Longitudinal Care Clinic is held twice a month at the R. Keith Summey Medical Pavilion in North Charleston.

To refer a patient or if you have any questions about the Fontan Longitudinal Care Clinic, contact Frances Woodard, CPNP by email or by phone 843-792-3292.

More Care Team Members

Pediatric Heart Failure/Heart Transplant

Vanessa Adams, PNP
Ali Burnette, PNP
Heather Henderson, M.D.

Palliative Care

Weston Rice, ACNP
Joan Cain, FNP