Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). In hypoplastic left heart syndrome, the left side of the heart is critically underdeveloped. If your baby is born with hypoplastic left heart syndrome, the left side of the heart can't effectively pump blood to the body, so the right side of the heart must pump blood to the lungs and to the rest of the body.There is no known cause in the majority of HLHS cases.There is likely a genetic component, as HLHS has been shown to be heritable and associated with specific gene mutations. 

Hypoplastic left heart syndrome is treated through several surgical procedures or a heart transplant. Your doctor will discuss with you the treatment options for your child. If the diagnosis has been made before the baby is born, doctors usually recommend delivery at a hospital with a cardiac surgery center. Medication to prevent closure of the connection (ductus arteriosus) between the right and left sides, followed by either surgery or a heart transplant, is necessary to treat hypoplastic left heart syndrome. 

Researchers actively study new treatments and surgeries for children who have hypoplastic left heart syndrome and other congenital heart diseases. Ongoing research includes studies in imaging and outcomes, human genetics and regenerative medicine strategies. Mayo doctors are also assembling a biorepository of patient and family histories, heart images, tissue and cell samples, and genomic information of people with hypoplastic left heart syndrome and their family members to further study the condition.

Of 370 cases entered, 245 underwent FCI: 100 aortic valvuloplasties from a previous single-center report (excluded from additional reporting here), an additional 86 aortic and 16 pulmonary valvuloplasties, 37 atrial septal cases, and 6 unclassified cases. FCI did not appear to affect overall survival to hospital discharge. Among live-born infants with a fetal diagnosis of aortic stenosis/evolving hypoplastic left heart syndrome, more than twice as many were discharged with biventricular circulation after successful FCI versus those meeting institutional criteria but without any or successful FCI (42.8% vs. 19.4%, respectively).