More than 85 percent of HLHS patients treated at University of Michigan Health survive to discharge, making C.S. Mott Children’s Hospital a worldwide leader in the treatment of HLHS. Advances in technology have led to increased ability to diagnose HLHS in infants before birth. 

At the Fetal Diagnosis & Treatment Center, maternal fetal medicine specialists (doctors who specialize in caring for high risk pregnancies) work in close collaboration with the fetal heart specialists from our congenital heart center to provide unparalleled prenatal diagnosis and treatment alternatives for mothers of infants diagnosed with HLHS. 

If you have questions about the fetal heart program at C.S. Mott Children’s Hospital, would like to schedule an appointment or if we can be of further assistance in any way, please call 1-877-475-6688.

Prenatal ultrasound can identify a problem with the baby's heart. The diagnosis of HLHS is made by fetal echocardiogram as early as 16 weeks gestation.  Due to the high number of mothers referred to our fetal heart center from throughout the nation, our fetal heart specialists have an unparalleled level of experience accurately diagnosing HLHS prenatally.

If there is a family history of congenital heart disease or if concern is raised during a routine ultrasound, the family is referred to a pediatric cardiology center where a detailed study of the heart is performed. 

Most newborns with HLHS have mild heart-related symptoms until the patent ductus arteriosus closes, usually within 48 hours of birth. Prior to closure of the ductus arteriosus, the baby's lips or fingernails may look slightly blue, especially when the baby cries. Babies with HLHS often breathe fast, have low energy levels, and/or have feeding problems. These changes can be subtle and can be difficult to detect in a newborn baby. When the ductus arteriosus does close, the baby may get very ill very quickly and develop symptoms of shock.

For babies diagnosed prenatally, in a small number of cases, fetal intervention for HLHS may be beneficial for babies with a restrictive atrial septal defect. These treatments are not a "cure," however, and children with HLHS will need expert medical attention for the rest of their lives.

For babies with HLHS, treatment generally consists of a three-staged surgical approach, or heart transplantation.

For children who are candidates for surgery, treatment usually consists of three heart operations done during the first two years of life. The goal of these operations is to rebuild the heart so that the right side can be used to pump blood out to the body.

Unless there is a special birth plan, a vaginal delivery is recommended and a cesarean section is reserved for obstetric indications. It is recommended that the delivery is planned at a hospital that is prepared for high-risk deliveries and also includes a neonatal intensive care, pediatric cardiology and pediatric cardiothoracic services. This will help facilitate coordination of care between specialties and permit access to other emergency services if needed. 

U-M Health is one of only a few centers nationwide at which the birth center is co-located within a comprehensive children’s hospital.  This unique setting allows for seamless integration between our private-room birth center, state-of-the-art newborn intensive care unit (NICU), and pediatric cardiothoracic intensive care unit in one convenient location.  

Future pregnancy

The majority of HLHS cases are due to a random (sporadic) event; however, in some families, there is an increased risk for future affected pregnancies. The approximated genetic risk for siblings of a child with isolated HLHS is 3%. If a chromosome change is identified, the chance of HLHS with future pregnancies is dependent on the type of chromosome change. Prenatal diagnostic testing may be available in some cases. Your doctor and a genetic counselor will meet with you and discuss your family's risk.  

• What is hypoplastic left heart syndrome (HLHS)?
• Prenatal care for a baby with hypoplastic left heart syndrome (HLHS)
• Overview of fetal cardiac interventions
• Fetal intervention for HLHS with a restrictive atrial septal defect (ASD)
• Delivery of a baby with hypoplastic left heart syndrome (HLHS)
• Why is genetic counseling important?