HLHS and Single Ventricle Defects

Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that requires a series of surgeries in a baby’s first few years of life. It can feel like a long road for families when they receive an HLHS diagnosis, but with expert care, children and their families can manage the condition.

Joe DiMaggio Children's Hospital Heart Institute has the experience and resources to diagnose HLHS in utero (before birth). We have successfully treated babies born with HLHS, and we provide lifelong follow-up care.

What Is HLHS?

HLHS is a type of congenital heart defect that affects 1 out of every 4,344 babies born in the United States, according to the Centers for Disease Control and Prevention (CDC). With HLHS, the left side of a baby’s heart is underdeveloped. The left ventricle is too small, and the heart valves and aorta aren’t formed correctly (or at all). Because of this, the left side of a baby’s heart cannot pump blood to the body properly.

We treat babies born with HLHS immediately to increase blood flow to the body. Through a series of surgeries, we bypass the left side of the heart so that the right ventricle can do the work of the left. This is why HLHS is called a single ventricle defect: one ventricle has to do the work of two.

Staged Heart Reconstruction Surgeries for HLHS

Our experienced surgeons provide the following three-staged reconstruction surgeries to treat HLHS. These surgeries gradually help restore the heart’s function so children can grow and thrive.

  • Norwood procedure (stage 1): Within 2 weeks of birth, we do this surgery to create the structures within the heart your baby needs. After the surgery, we’ll monitor your baby very carefully. Follow-up care may include medicine to help your child’s heart work better.
  • Bi-directional Glenn shunt procedure (stage 2): We perform this surgery between 4 and 6 months of age. This surgery re-routes how blood flows. A nurse practitioner and your physician will closely monitor your baby throughout this stage.
  • Fontan procedure (stage 3): This is the final surgery, which we do between the ages of 18 months and 3 years. We make the final connections between the vein and the blood vessel in the lungs. It’s very important to continue regular follow-ups with a cardiologist after all surgeries are complete.

How We Care for Children With HLHS and Other Single Ventricle Defects

We manage children with HLHS from birth through adulthood, offering:

  • Prenatal management: Using fetal echocardiogram, we can diagnose HLHS as early as 20 weeks of pregnancy. We then guide families during pregnancy, delivery, and the first few days of life until the time of surgery. You’ll get answers to all of your questions and guidance through your delivery so that you don’t have to seek consultation elsewhere. Learn more about fetal diagnosis and management.
  • Specialized surgical team: Our pediatric cardiac surgeons are ready to treat newborns with HLHS or other single ventricle conditions. We have an entire support team with specialized experience in caring for babies with HLHS. Learn more about heart surgery.
  • Interstage monitoring: The time between stage 1 and stage 2 surgeries is a high-risk time for infants and a scary one for parents. We monitor babies and provide careful follow-up to help babies stay strong and ready for the next surgery. With our Single Ventricle and High-Risk Home Monitoring Program, patients and their families are never too far from a medical professional who can provide guidance or answer questions should problems arise. This program keeps you in touch with:
    • Our experienced nurse practitioners
    • In-house cardiac specialists
    • An extensive network of community cardiologists
  • Care in our cardiac ICU: We have a dedicated intensive care unit staff with experience in taking care of HLHS babies. With each surgery, your child will be in excellent and caring hands as they recover in the cardiac ICU. Read about our cardiac ICU.
  • Follow-up care: We love watching babies grow and thrive. Our adult congenital heart disease program allows us to care for people of all ages as your lifelong partners in healthy hearts.
  • Heart transplant: For children with HLHS who need a heart transplant, Joe DiMaggio Children’s Hospital is an accredited pediatric heart transplant center. We have excellent heart transplant outcomes, and an entire team devoted to caring for these children. Learn more about heart transplant.

Contact Us

To schedule an appointment or learn about our award-winning pediatric heart team, call 954-265-3437.