Hypoplastic Right Heart Syndrome Treatment

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The day your baby is born is cause for celebration. But then, an emergency: your baby is turning blue. They aren’t breathing right. They’re not getting the oxygen they need, which is a life-threatening situation. It could be from hypoplastic right heart syndrome.

Sometimes, hypoplastic right heart syndrome is found before birth. Your doctor might give you a special ultrasound (fetal echocardiogram) to check your baby’s heart. This ultrasound can show your baby's heart issues before your baby is born.

We’re here to help your baby. At UVA Health, we treat the hardest children’s heart cases. As the #1 children’s hospital in Virginia, we specialize in caring for babies just like yours. We’re specially trained in treating complex heart cases in children. And we have proven experience and success in treating hypoplastic right heart syndrome.

Treating Hypoplastic Right Heart Syndrome at UVA Children’s

Hypoplastic right heart syndrome (HRHS) is a type of heart issue your baby is born with (congenital heart defect). Your baby’s heart didn’t fully form on the right side. Your baby might have a missing valve in their heart  (called either tricuspid atresia or pulmonary atresia, depending on the affected valve). This means a smaller and weaker right heart. That prevents your baby’s blood from having normal oxygen levels. Our children’s heart care team can help.

Surgery can help your baby. It’s a three-step process that happens over time. The three surgeries change the way blood flows in and around your baby’s heart.

  • First, they’ll have a small tube put in that delivers blood flow to their lungs. Called a BT shunt (Blalock-Taussig shunt), this happens in their first week of life.
  • At around 4 to 6 months of age, they’ll have a Glenn procedure. This directly connects a major vein (superior vena cava) and their pulmonary artery. This lets oxygen-poor blood from the upper body go straight to the lungs to get oxygen. (It’s also sometimes called the bidirectional Glenn procedure.)
  • Between ages 2 and 4, they’ll have a Fontan procedure. This connects another major vein (inferior vena cava) and the pulmonary artery. This lets oxygen-poor blood from the lower body get oxygen directly from the lungs.

Without surgery, your baby’s organs won't get the right amount of oxygen. This could cause them to fail. But with surgery, we can help your baby’s heart work better. They’ll be able to thrive.

What future care for your child looks like depends on how well they do after the Fontan procedure. Rarely, some people with HRHS need a heart transplant later in life. 

What’s Hypoplastic Right Heart Syndrome?

Hypoplastic right heart syndrome is rare. The right side of your baby’s heart didn’t form as it should while they were growing in the womb. No one knows why they’re born with this condition.

Hypoplastic right heart syndrome can affect different parts of your baby’s heart, like:

  • Tricuspid valve (controls blood flow between the top and bottom heart chambers) may not open
  • Pulmonary valve (controls blood flow going to the lungs) may not form or open 
  • Pulmonary artery (takes blood from the right side of the heart to the lungs) may be too small
  • Right ventricle (bottom heart chamber) doesn't fully develop

The tricuspid and pulmonary valves might be too small, completely blocked, or are absent. Sometimes, the valves didn’t open.

What’s Tricuspid Atresia?

The tricuspid valve controls blood flow between the top and bottom chambers of the right heart. Tricuspid atresia is when the tricuspid valve doesn’t form. This prevents blood from getting into the right ventricle and pulmonary artery. It also causes the right ventricle to be too small and not work correctly.

What’s Pulmonary Atresia?

The pulmonary valve controls blood flow between the bottom right chamber and the lungs. Pulmonary atresia is when the pulmonary valve doesn’t form. Blood must get to the lungs so that your baby has enough oxygen in their blood. To correct it, we make a new route for the blood to get to the lungs.

Diagnosing Hypoplastic Right Heart Syndrome

HRHS can be seen on a fetal echocardiogram before your baby is born. This special ultrasound shows how your baby’s heart is developing in the womb. We can see how it’s forming and if it’s working right.