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PubMed This is a summary of 29 peer-reviewed journal articles Updated
Pediatric Cardiology

The Roadmap of Care: Navigating Treatment Pathways for HRHS

At a Glance

Treatment for Hypoplastic Right Heart Syndrome (HRHS) depends on the size and function of the right ventricle. Doctors choose either a biventricular repair (a two-pump system) if the right ventricle can grow, or a single-ventricle palliation to let the left ventricle do all the work.

The treatment of Hypoplastic Right Heart Syndrome (HRHS) is not a “one-size-fits-all” approach. Instead, it is a tailored roadmap based on the size and shape of your child’s right ventricle. Doctors generally choose between two primary paths: Biventricular Repair (two-pump system) or Single-Ventricle Palliation (one-pump system) [1][2].

Fetal Interventions: Starting Early

In some cases, treatment can begin before birth. Fetal Pulmonary Valvuloplasty (FPV) involves using a needle through the mother’s abdomen to open the baby’s pulmonary valve [3][4]. The goal is to improve blood flow and encourage right ventricle growth during the remainder of the pregnancy, potentially making a biventricular repair more likely after birth [4][5].

Immediate Steps: Life After Birth & The CICU

Before any major surgery, the first goal is to ensure your baby is stable and getting enough oxygen to their blood.

What to Expect in the CICU: Immediately after birth, your baby will likely be moved to the Cardiac Intensive Care Unit (CICU). You will see them hooked up to multiple IV lines, monitors, and potentially a breathing tube (ventilator). This visual shock is completely normal to feel overwhelmed by. During stabilization, depending on your baby’s stability, you may be able to gently touch or hold them, but always ask the CICU nurses first [6][7].

  • Prostaglandin E1 (PGE1): This medication is started immediately after birth via IV to keep the ductus arteriosus (a natural fetal blood vessel) open. This vessel acts as a temporary “bridge” to get blood to the lungs [8][9].
  • Initial Stabilization: To provide a more permanent bridge than medication, doctors may use:
    • Ductal Stenting: A tiny mesh tube (stent) is placed in the ductus arteriosus via a catheter. This often leads to shorter hospital stays compared to surgery [10][11].
    • BTT Shunt (Blalock-Taussig-Thomas Shunt): A surgically placed tube that connects a major artery from the body to the pulmonary artery, creating a permanent pathway for blood to reach the lungs [12][13].

The Two Treatment Pathways

The choice of pathway depends heavily on whether the right ventricle is “pump-capable.”

Feature Path A: Biventricular Repair (Two-Pump) Path B: Single-Ventricle (One-Pump)
Goal Right ventricle pumps to lungs, Left to body Left ventricle pumps to both lungs and body
Typical Anatomy Tripartite RV, growing Tricuspid Valve Very small RV, RVDCC present
Timeline Interventions at birth, ongoing growth monitoring Staged surgeries (Birth, 4-6 months, 2-4 years)

Path A: Biventricular Repair (The Two-Pump Goal)

This path is chosen if the right ventricle has the potential to grow and eventually pump blood to the lungs on its own [14].

  • Who Qualifies? Typically babies with a tripartite right ventricle (all three parts present) and a tricuspid valve Z-score generally above -2 or -3 [1][2].
  • The Procedure: Doctors may perform a pulmonary valve perforation or valvuloplasty using a catheter soon after birth to open the blocked valve and let blood flow into the right ventricle, encouraging it to grow [15][16]. Over the first few months of life, further procedures may be evaluated based on growth.
  • 1.5 Ventricle Repair: A “middle-ground” option where the right ventricle pumps some blood, but a Glenn procedure is also done to assist it [17].

Path B: Single-Ventricle Palliation (The Staged Path)

If the right ventricle is too small to ever function as a pump, the goal shifts to making the left ventricle do the work for the whole body [18][19].

  1. Stage 1 (Neonatal): Stabilization via a BTT shunt or ductal stent to ensure blood reaches the lungs [10][12].
  2. Stage 2 (The Glenn): Usually performed at 4–6 months. The superior vena cava (which brings blood from the upper body) is connected directly to the pulmonary arteries [20][21].
  3. Stage 3 (The Fontan): Usually performed between ages 2 and 4. The inferior vena cava (from the lower body) is connected to the pulmonary arteries. After this, all oxygen-poor blood flows directly to the lungs without needing a pump [18][22].

Making the Decision

Your care team will use a “decision tree” approach:

  • Is there RVDCC? (Right Ventricle-Dependent Coronary Circulation): If yes, a biventricular repair is usually too dangerous, and the single-ventricle path is chosen [23][24].
  • Is the RV tripartite? If yes, the team is more likely to attempt a biventricular repair [25][1].
  • Is the Tricuspid Valve growing? If the valve grows after the first procedure, the team may continue toward a full repair [26][27].

While the path may change as your child grows, the ultimate goal remains the same: ensuring the most efficient and stable circulation possible for your child’s unique heart [28][29].

Common questions in this guide

What is the difference between biventricular and single-ventricle repair for HRHS?
Biventricular repair aims to use both sides of the heart, allowing the right ventricle to eventually pump blood to the lungs on its own. Single-ventricle repair is used when the right ventricle is too small, relying on a series of surgeries to let the left ventricle pump for the entire body.
What happens immediately after a baby with HRHS is born?
After birth, your baby will be moved to the Cardiac Intensive Care Unit (CICU) for stabilization. They will likely receive a medication called Prostaglandin E1 through an IV to keep a fetal blood vessel open, which ensures blood can reach the lungs until a more permanent procedure is done.
How do doctors decide which surgery path is best for my baby's heart?
The decision depends entirely on the size, shape, and growth potential of your child's right ventricle. If the right ventricle has all three anatomical parts and the tricuspid valve is growing, doctors will lean toward a biventricular repair.
What is a BTT shunt or ductal stent used for in HRHS?
Both are initial procedures used to secure a stable pathway for blood to reach the lungs. A stent is a tiny mesh tube that props open an existing fetal blood vessel, while a BTT shunt is a surgically placed tube that creates a new bridge from a major artery to the lungs.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my child's anatomy, are we aiming for a biventricular repair, a 1.5-ventricle repair, or the single-ventricle (Fontan) pathway?
  2. 2.What are the risks and benefits of ductal stenting versus a surgical Blalock-Taussig-Thomas (BTT) shunt for my baby?
  3. 3.If we are pursuing a biventricular path, how will you monitor the right ventricle's growth over time?
  4. 4.Can you explain the specific criteria (like tricuspid valve size) that would cause us to switch from a biventricular plan to a single-ventricle plan?
  5. 5.How many of these specific procedures (like pulmonary valve perforation or the Glenn) does this surgical team perform annually?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

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This page provides educational information about HRHS surgical pathways and care. It does not replace professional medical advice. Always consult your pediatric cardiologist and surgical team to determine the best treatment plan for your child's unique heart anatomy.

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