Hypoplastic Left Heart Syndrome (HLHS) Surgery

With hypoplastic left heart syndrome (HLHS), a baby is born with an abnormality of the heart or heart valves that results in the left side of the heart being smaller and weaker than it should be. The left side of the heart can’t do its job pumping blood through the body. HLHS surgery is required to repair the condition.

As the leading providers of pediatric heart care in Louisville and Southern Indiana, our specialists are experienced with successfully treating hypoplastic left heart syndrome.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, have the skill and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.

Since the left side of the heart doesn’t work correctly, the right side of the newborn’s heart has to work harder, and there has to be a connection between the right and left sides of the baby’s circulation so blood can get to the only functioning ventricle.

Babies born with HLHS need an atrial septal defect — a hole between the right and left chambers of the heart. Babies born with HLHS but without this hole will need an emergency intervention called a balloon septostomy shortly after birth to create such a hole.

The atrial septal defect and a normal opening in a newborn’s heart — the patent ductus arteriosus (PDA)— allow the heart to pump blood to both the lungs and throughout the body. Normally, the PDA shrinks and closes during the first few days of life. In a baby with HLHS, the PDA must stay open so blood can flow to the body. Prostaglandins are a medication frequently given to babies with HLHS after birth to keep the PDA open.

The cardiothoracic specialists at Norton Children’s Heart Institute are trained to act swiftly to keep the PDA and the ASD open until the heart is repaired with HLHS surgery.

HLHS surgery is a complex series of procedures that require skill and expertise

Hypoplastic Left Heart Syndrome Diagnosis

HLHS may be seen on ultrasound scans during pregnancy. A fetal echocardiogram provides more information and tells the delivery team how to prepare for care after birth.

Common tests used to learn more about a newborn’s heart include:

HLHS Surgery

HLHS most often is diagnosed through ultrasound before the baby’s birth, allowing the cardiothoracic specialists at Norton Children’s Heart Institute to prepare and move swiftly to stabilize the newborn. Babies born showing the first signs of HLHS will be stabilized immediately while more diagnostic tests are performed.

One of the first treatments is a medication called prostaglandin that is used to keep the patent ductus arteriosus open. Doctors will use medications and mechanical ventilation strategies (breathing assistance) to balance how much blood flows to the body and how much goes to the lungs.

In the past, this condition often was considered inoperable. Today, HLHS surgery may involve a series of three surgeries. In some cases, a child with hypoplastic left heart syndrome may need a heart transplant because the case is so complex or because the heart becomes weak after HLHS surgery.

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The three surgeries for HLHS are:

The Norwood Procedure

  • This surgery is typically done in the first week of a baby’s life.
  • Cardiothoracic surgeons create a new aorta to connect to the right ventricle.
  • A tube is placed from either the large arteries off the aorta or the right ventricle and connected to the blood vessels supplying the lungs, also known as the pulmonary arteries.
  • The right ventricle is then able to pump blood to both the lungs and the rest of the body.

The Glenn Procedure

  • This surgery is typically performed when the infant is 4 to 6 months old.
  • Cardiothoracic surgeons create a direct connection between the pulmonary artery and the superior vena cava, the blood vessel that returns oxygen-poor blood from the upper part of the body to the heart.
  • The procedure reduces the work of the right ventricle by allowing blood returning from the body to flow directly to the lungs.

The Fontan Procedure

  • This surgery is typically performed when the child is 2 to 4 years old.
  • Cardiothoracic surgeons connect the pulmonary artery and the inferior vena cava, the blood vessel that returns oxygen-poor blood from the lower part of the body to the heart.
  • This procedure allows the rest of the blood coming back from the body to go to the lungs.
  • Once this procedure is complete, oxygen-rich and oxygen-poor blood will no longer mix in the heart and the child’s skin will no longer look bluish.
  • Some patients will have a “fenestration” (opening) connecting the venous and arterial circulations, which allows mixing to periodically occur.

These surgeries don’t cure HLHS. The child will need regular follow-up visits with a pediatric cardiologist to monitor progress. When the child becomes an adult, they will need to see an adult congenital heart disease (ACHD) specialist to provide care.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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