Hypoplastic Left Heart Syndrome (HLHS)

What is hypoplastic left heart syndrome?

Hypoplastic left heart syndrome (HLHS) is a condition in which the structures of the left side of the heart are underdeveloped and unable to properly support circulation to the body. HLHS is one of the most common of the congenital heart defects, occurring in approximately one to four out of 10,000 live births.

How is this condition managed during pregnancy?

When HLHS is suspected, further evaluation includes a specialized evaluation of the fetal heart (fetal echocardiogram). As HLHS is frequently associated with other physical abnormalities and genetic syndromes, genetic counseling and testing are recommended. Prenatal consultations with pediatric cardiology, pediatric cardiothoracic surgery, and neonatology specialists are recommended to discuss the prognosis and management after birth. Prenatal care will be managed by a maternal-fetal medicine specialist, an obstetrician with special training and expertise in high-risk pregnancies.

How is this condition managed after delivery?

Newborns with HLHS will be evaluated and cared for by a team involving pediatric cardiology and neonatology specialists. Surgical palliation (non-curative treatment to enable survival) involves three operations spaced out over the early years of life, with the first surgery taking place within the first week after birth.