Hypoplastic Right Heart Syndrome

What is hypoplastic right heart syndrome?

Hypoplastic right heart syndrome is a rare heart issue that some babies have at birth (congenital). Parts of the right side of their hearts don’t develop fully with HRHS. This causes a low level of oxygen in their blood.

Babies with hypoplastic right heart syndrome may have issues with these parts of their heart:

• Tricuspid valve.
• Pulmonary valve.
• Right ventricle.
• Pulmonary artery.

Depending on whether the condition is severe or mild, these heart structures may not work as they should.

Babies with HRHS may also have another heart issue, like:

• Atrial septal defect (hole in the wall between your heart’s two upper chambers).
• Ventricular septal defect (hole in the wall between your heart’s two lower chambers).
• Atrioventricular canal defect (a hole in the wall in the center of your heart, plus other issues).

How serious is hypoplastic right heart syndrome?

Hypoplastic right heart syndrome can be more serious for some people than for others. People who have severe issues that cause low oxygen levels need treatment right away. Other people with HRHS have milder issues and don’t get a diagnosis until adulthood.

How common is hypoplastic right heart syndrome?

Researchers estimate that fewer than 50,000 people in the United States have an HRHS heart. It’s rarer than hypoplastic left heart syndrome.

What are the symptoms of hypoplastic right heart syndrome?

Hypoplastic right heart syndrome symptoms may include:

• Blue skin on your hands and feet (cyanosis).
• Shortness of breath (dyspnea).
• Rapid breathing (tachypnea).
• Not feeding well.
• Fatigue.

What causes hypoplastic right heart syndrome?

The cause of hypoplastic right heart syndrome is unknown.

Researchers believe genetics could be involved in causing hypoplastic right heart syndrome. They’re looking into this because more than one person in the same family has had HRHS at birth.

What are the complications of hypoplastic right heart syndrome?

Hypoplastic right heart syndrome can lead to:

• Low oxygen level (hypoxemia).
• Right-sided heart failure.
• Abnormal heart rhythms (arrhythmias)

As young adults, people with mild HRHS can have miscarriages because of low oxygen levels.

How is hypoplastic right heart syndrome diagnosed?

A healthcare provider can diagnose hypoplastic right heart syndrome during fetal development. They can use ultrasound (which is noninvasive) to see the fetus’s heart. A fetal echocardiogram (also noninvasive) can give providers a better look at a fetus’s heart.

What tests will be done to diagnose hypoplastic right heart syndrome?

After birth, providers can use these noninvasive tests to diagnose HRHS:

• Pulse oximeter (pulse ox, a monitor that measures your oxygen level).
• Electrocardiogram (EKG).
• Echocardiogram (echo).
• Heart MRI (magnetic resonance imaging).

How is hypoplastic right heart syndrome treated?

Hypoplastic right heart syndrome treatment may include medicine, minimally invasive procedures or surgery. The goal of these treatments is to help blood get to your baby’s lungs so they can get more oxygen into it.

Medicine

Prostaglandin can help keep a baby’s ductus arteriosus open. This is a blood vessel a fetus uses to get oxygen-rich blood from its birth parent. After birth, a baby can use its lungs to get oxygen instead. Usually, a ductus arteriosus closes a few days after birth. Keeping this route open after birth can help blood get to a baby’s lungs. HRHS prevents the usual route from working.

Minimally invasive procedures

A provider can use a catheter to place a stent (small metal tube) inside a baby’s ductus arteriosus. This can keep it open for better blood flow to a baby’s lungs.

A healthcare provider can perform a pulmonary valve perforation. They can thread a catheter wire through a blood vessel to get to the pulmonary valve that doesn’t open as it should. Then they make a hole in the pulmonary valve to let blood flow through to the pulmonary artery. Some providers use ablation for this procedure.

Surgery

Operations can treat severe cases of hypoplastic right heart syndrome.

A Blalock-Taussig-Thomas (BTT) shunt can establish blood flow to the lungs in a baby with an HRHS heart. It uses synthetic fabric to link their subclavian artery to their pulmonary artery.

Months or years later, a surgeon may perform a Glenn procedure and/or Fontan procedure. Each of these operations creates a new route for blood to flow around the right ventricle (lower heart chamber) that isn’t working well. A fully developed right ventricle can pump blood to your pulmonary artery.

The Glenn and Fontan procedures allow blood to flow from a major vein to your pulmonary artery. From there, blood flows to your lungs, where it can get oxygen.

Complications of the treatment

Complications can vary by procedure, but may include:

• Stents can move out of place.
• A BTT shunt can cause blood vessel narrowing or a nerve injury.
• A pulmonary valve perforation can cause abnormal heart rhythms (arrhythmia) or injury to a blood vessel.
• Surgery complications can include high blood pressure, pleural effusions, cardiac arrest, kidney or liver disease or abnormal heart rhythms.

How long does it take to recover from treatment?

Recovery time varies by procedure. Surgeries have the longest recovery. Your child could be in the hospital for several days — even more than a week.

What can I expect if my baby has hypoplastic right heart syndrome?

Because of a low level of oxygen early in life, people who have hypoplastic right heart syndrome may face challenges like:

• Language issues.
• Memory issues.
• Higher risks of heart, liver or kidney issues in adulthood.
• Mood or anxiety disorders.
• Difficulty with planning or problem-solving.

What is the prognosis for HRHS?

It’s rare for people with severe issues from hypoplastic right heart syndrome to survive into adulthood without surgery. People with mild HRHS may not even realize they have it until adulthood.

How do I take care of my child with HRHS?

The care your baby needs will depend on the severity of their condition. If they need surgery soon after birth, you’ll need to monitor them closely once they’re home. Your provider can show you how to care for your child.

When should my child see a healthcare provider?

A baby who has surgery will need long-term follow-up appointments with a provider. They can tell you what to watch for in your child and what you should do. If you have a question or concern about your child’s condition, contact their provider.

If your child has issues with learning or with handling their emotions, they may need to see other providers who can help them with those areas. Their provider can refer you to specialists who can focus on your child’s needs.

What questions should I ask my doctor?

Questions to ask your child’s provider may include:

• Does my child need surgery?
• How soon do they need surgery?
• How many surgeries will they need and when?
• What’s the outlook for my child’s specific situation?

A note from Cleveland Clinic

Learning that your child has a heart condition is difficult. But children born today have access to treatments that didn’t exist for previous generations. This is the time to be an advocate for your child. Learn about HRHS and ask questions about anything that isn’t clear. Talk to your child’s provider to reach the best decisions for them.