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PubMed This is a summary of 101 peer-reviewed journal articles Updated
Pediatric Cardiology

Hypoplastic Right Heart Syndrome (HRHS): A Parent's Guide

At a Glance

Hypoplastic Right Heart Syndrome (HRHS) is a congenital heart defect where the right side of the heart is underdeveloped. Treatment is highly individualized, involving either biventricular repair or single-ventricle surgeries like the Fontan procedure, requiring lifelong specialized cardiac care.

Receiving a diagnosis of Hypoplastic Right Heart Syndrome (HRHS) is overwhelming. This guide is designed to empower you with the knowledge needed to understand your child’s condition, decode complex medical jargon, and partner effectively with your care team. Every child’s heart is unique, and this resource will help you navigate the journey ahead.

Here is what you will find in this guide:

01

Orientation and Validation: Your Child's HRHS Diagnosis

Learn what a Hypoplastic Right Heart Syndrome (HRHS) diagnosis means for your baby. Understand the next steps, stabilizing treatments, and surgical plans.

02

Anatomy and Subtypes: Mapping Your Child's Heart

Understand the anatomy of Hypoplastic Right Heart Syndrome (HRHS). Learn about PA-IVS, Tricuspid Atresia, RVDCC, and how the defect is diagnosed in babies.

03

Deciphering the Report: Interpreting Echocardiograms and Z-Scores

Learn how to read your child's HRHS echocardiogram report. Understand what Z-scores, tricuspid valve annulus measurements, and right ventricle morphology mean.

04

The Roadmap of Care: Navigating Treatment Pathways for HRHS

Learn about Hypoplastic Right Heart Syndrome (HRHS) treatment pathways. Understand biventricular repair, single-ventricle palliation, and immediate newborn care.

05

Life After the Fontan: Navigating Long-Term Health and Monitoring

Learn what to expect after the Fontan procedure. Understand liver disease risks, PLE, exercise limits, and how to navigate lifelong health monitoring.

06

Building Your Care Team and Preparing for Visits

Learn how to build a multidisciplinary care team for Hypoplastic Right Heart Syndrome (HRHS). Discover what to bring to visits and how to choose a center.

Common questions in this guide

What are the treatment options for a child with HRHS?
Treatment depends on the specific anatomy of your child's heart. Specialized care teams typically explore either a biventricular pathway to utilize both ventricles, or a single-ventricle pathway that often concludes with the Fontan procedure.
How do I read my child's HRHS echocardiogram report?
Echocardiogram reports frequently use measurements called Z-scores to compare the size of your child's heart structures to a healthy heart of the same body size. Your pediatric cardiologist uses these numbers to determine the best surgical and treatment approach.
What does long-term care look like after the Fontan procedure?
After completing the single-ventricle pathway, your child will need lifelong monitoring by a specialized congenital cardiologist. Regular check-ups are essential to ensure their unique heart circulation continues to function well as they grow.
What questions should I ask my child's HRHS care team?
It is crucial to ask who will be your primary point of contact for coordinating care across different specialties. You should also regularly review the treatment roadmap with the team and ask about available mental health and family support resources.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Who will be the primary point of contact for coordinating my child's care across different specialties?
  2. 2.How frequently will we review the overall treatment roadmap as my child grows?
  3. 3.What mental health and family support resources does your center offer for parents of children with complex congenital heart disease?

Questions For You

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This guide provides educational information about Hypoplastic Right Heart Syndrome for parents and caregivers. It does not replace professional medical advice, so always consult your pediatric cardiologist regarding your child's specific diagnosis and treatment plan.

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