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PubMed This is a summary of 28 peer-reviewed journal articles Updated
Pediatric Cardiology

Building Your Care Team and Preparing for Visits

At a Glance

Managing Hypoplastic Right Heart Syndrome (HRHS) requires a multidisciplinary team including pediatric cardiologists, surgeons, and neurodevelopmentalists. Choosing a high-volume center with specialized programs and organizing a master folder of medical records improves your child's long-term care.

Managing Hypoplastic Right Heart Syndrome (HRHS) is a long-term commitment that requires more than just a single cardiologist. Because this condition can affect multiple systems in the body—including the liver, brain, and kidneys—a multidisciplinary care team is essential [1][2]. Building the right team early, especially at a high-volume center, can significantly improve your child’s long-term outcomes and quality of life [3][4].

Your Care Team Roster

A comprehensive “Heart Team” for HRHS should include specialists who address both the immediate heart defect and the secondary impacts on the body [1][5].

  • Pediatric Cardiologist: Your primary “quarterback” for day-to-day heart management.
  • Congenital Heart Surgeon: Performs the staged repairs or biventricular conversions [6].
  • Electrophysiologist (EP): A cardiologist who specializes in heart rhythms and managing arrhythmias [7].
  • Hepatologist: A liver specialist who monitors for Fontan-Associated Liver Disease (FALD) [8][9].
  • Cardiac Neurodevelopmentalist: Tracks milestones and academic development, as children with complex heart defects are at higher risk for learning challenges [10][11].
  • Maternal-Fetal Medicine (MFM): For those with a prenatal diagnosis, an MFM specialist coordinates the birth and early stabilization plan [12][13].

Choosing a High-Volume Center

Research consistently shows that children with rare, complex heart defects often have better outcomes at centers that perform a high volume of these procedures [14][15]. When vetting a center, look for:

  1. Risk-Adjusted Outcomes: Ask for their data using standard scoring systems like the Society of Thoracic Surgeons (STS) database or RACHS-1 [16][17].
  2. Specialized Programs: Does the center have a specific “Biventricular Repair” program or a “Fontan Clinic”? [18][4].
  3. Advanced Technology: High-volume centers often use 3D echocardiography, cardiac MRI, and even 3D-printed heart models to plan surgeries for complex anatomy [19][20].

Preparing for Your First Visit

Whether you are moving care to a new center or preparing for your child’s first post-birth consultation, being organized is your best tool for advocacy [21][22].

The Essential Document Checklist

Do not rely on hospitals to send records to each other; always bring your own “Master Folder” containing [23][24]:

  • [ ] Imaging Disks: Digital copies of all echocardiograms, CT scans, and MRIs on CD or USB.
  • [ ] Surgical Reports: Detailed notes from every heart procedure your child has had.
  • [ ] Discharge Summaries: Narratives from every hospital stay.
  • [ ] Medication List: Including exact dosages and the time of the last dose.
  • [ ] Growth Charts: Information on your child’s weight and height trends.

The Logistics of Transferring Care

If you need to move your child to a more specialized facility, the process requires careful coordination.

  • Specialized Transport: Critically ill infants may need a dedicated transport team with specialized ventilators or even mobile ECMO (heart-lung bypass) capabilities [25][26].
  • Inter-Facility Communication: Ensure your current team has spoken directly to the receiving team to minimize “gaps” in care during the transition [23][27].

By assembling a dedicated multidisciplinary team and keeping rigorous records, you ensure that your child’s care is consistent, well-informed, and focused on their whole-body health [21][28].

Common questions in this guide

Who should be on a care team for Hypoplastic Right Heart Syndrome?
A comprehensive HRHS care team should include a pediatric cardiologist, congenital heart surgeon, electrophysiologist, and hepatologist. It is also important to include a cardiac neurodevelopmentalist to track learning and developmental milestones.
What should I look for when choosing a hospital for my child's HRHS care?
Look for high-volume centers with specialized programs like a Fontan Clinic or Biventricular Repair program. It is highly recommended to ask for their risk-adjusted outcomes data using standard scoring systems like the STS database.
What medical records do I need to bring to an HRHS specialist visit?
You should bring a master folder containing digital copies of all imaging scans, such as echocardiograms and MRIs. You will also need surgical reports, hospital discharge summaries, growth charts, and an exact medication list.
Why does a child with a heart defect need to see a liver specialist?
Children with complex heart defects who undergo certain procedures are at risk for a complication called Fontan-Associated Liver Disease. A hepatologist monitors liver health to identify and manage any potential issues as early as possible.
How do I safely transfer my child's care to a specialized heart center?
Critically ill infants may require a specialized transport team equipped with advanced life support, such as mobile ECMO capabilities. Direct communication between the transferring and receiving doctors is vital to prevent any gaps in your child's care.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What is your center's specific annual volume for biventricular conversions and Fontan procedures?
  2. 2.Does your surgical team use advanced planning tools, like 3D-printed heart models or virtual reality, for complex HRHS cases?
  3. 3.Can you provide your center's risk-adjusted survival data for PA-IVS (Pulmonary Atresia with Intact Ventricular Septum)?
  4. 4.Do you have a dedicated Cardiac Neurodevelopmental Program for long-term follow-up?
  5. 5.Who will be the 'point person' on my child's multidisciplinary team for coordinating care between cardiology, hepatology, and neurology?

Questions For You

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References

References (28)
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This page provides educational information on building a care team for Hypoplastic Right Heart Syndrome. It does not replace professional medical advice from your child's pediatric cardiologist or surgical care team.

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