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Single Ventricle Journey: Children & Young Adults

In the Single Ventricle Center of Excellence, we follow patients at all points along their Roadmap with single ventricle physiology. After a child has their third palliative surgery (The Fontan Operation), our team of dedicated providers follows them in the Single Ventricle Follow-Up Clinic at the main hospital. Our team provides a flexible service model ranging along a spectrum from primary cardiology follow-up visits through “as needed” consultative services. Frequency of follow-up with our team is based on a collaborative discussion with the family and the referring cardiologist. Every patient will have their care managed by our nurse coordinator, an advanced practice provider, and a dedicated cardiologist.

Beyond specialized cardiac care, our team also works with other experts to monitor the other vital organs – for example routine liver evaluations, nutrition counseling, exercise & fitness testing, and neurodevelopmental and school support. In addition to watching for other organ issues, we know that the Fontan operation is often not the final intervention for these children. Many of them will require additional operations or catheter-based procedures in late childhood or young adulthood. They are also at risk for heart rhythm problems and progressive cardiac dysfunction. In some children and young adults, this may require heart transplantation. Children with single ventricle physiology are also at increased risk for a broad spectrum of neurodevelopmental and psychosocial issues that require careful surveillance throughout childhood in order to maximize their functional health outcomes and quality of life. Proper monitoring for these potential issues requires a dedicated team with specific expertise in Single Ventricle careIn order to best take advantage of the talents of our entire multidisciplinary team, all patients are collaboratively reviewed prior to their next clinic visit and/or with any major medical decisions to ensure that each patient’s short and long-term medical plan is optimized. 

Referrals

Patients living with single ventricle defects can be referred for a one-time evaluation, for alternating visits with their primary cardiologist, for periodic consultations prior to major procedures or transition periods, or for all of their cardiac care. Requests for evaluation can be made by inpatient care teams, outpatient cardiologists or by families themselves. Patients with single ventricle defects who are referred will undergo a comprehensive evaluation by our multi-disciplinary team in the Single Ventricle Center of Excellence 

First Visit

Prior to your first visit, all health records (medical, arrhythmia, and surgical history and reports, as well as cardiac images on CDs or DVDs) are reviewed by the single ventricle team before the patient comes to our center for evaluation. Additional subspecialty appointments are coordinated on an as needed basis. This may include a further consultation with a gastroenterologist, hepatologist, immunologist, endocrinologist or hematologist. Based on the patient’s age and history, additional testing such as echocardiogramHolter monitorblood work, cardiac MRI or CTcardiopulmonary stress test, cardiac catheterizationand intracardiac electrophysiology study may be performed at Lurie Children’s. 

Cardiovascular Bridge Program to Adult Care

For many teenage patients, the transition from pediatric to adult care can be confusing and frustrating, often resulting in gaps in treatment. For young adults with complex congenital heart disease, this can be particularly dangerous. Our Cardiovascular Bridge Program is designed to create a seamless transition to adult congenital care in Lurie Children’s Adult Congenital Heart Disease (ACHD) Program and next door at the Northwestern Medicine Bluhm Cardiovascular InstituteThis program facilitates collaboration between adult and pediatric cardiac specialists and ensures continuity of care as patients transition to independent adulthood. Designed for patients 16-26 years of age, the Cardiovascular Bridge Program allows pediatric patients to be seamlessly integrated into our ACHD Program which is designed to care for adult patients with congenital anomalies best cared for by pediatric specialists. Adult patients may also be referred to our Fontan Conversion and Arrhythmia Surgery ProgramThis internationally respected program has particular expertise in managing arrhythmias associated with prior Fontan procedures in adult patients. 

Make an Appointment

Appointments with the Single Ventricle Center of Excellence can be arranged by calling 312.227.8588.