HLHS is the abbreviation for Hypo-Plastic Left Heart Syndrome, a chronic pediatric congenital disease. It affects normal blood flow through the heart.  As the baby develops during pregnancy, the left side of the heart does not form correctly.  The CDC has estimated that each year in the US about 960 babies are born with this defect, about one in every 4,344 babies.

The prognosis for surviving this condition has improved significantly in recent years.  Children born with HLHS usually undergo a series of three open heart surgeries known as the Norwood, The Glenn, and the Fontan.  These surgeries are designed to create normal blood flow in and out of the heart so the body gets the oxygen-rich blood that it needs.  

The Norwood is usually performed within the first two weeks of life. Surgeons reconstruct the Aorta and connect it with a shunt directly to the right ventricle of the heart. This procedure allows the right ventricle to pump blood to both the lungs and the body.

The Glenn procedure is generally the second operation usually performed between three to six months of age. This procedure reduces the work of the right ventricle by allowing it to pump blood mainly to the the Aorta. 

The third operation is the Fontan usually performed between 18 months ad 4 years of age.  After this procedure, there is no mixing of oxygen rich blood and oxygen poor blood in the heart and the bluish cast to the child’s skin will subside and look more pink and healthy.

After these operations some medications may be needed to regulate heart and blood pressure functions. The cardiologist may also recommend limiting some physical activity.  With proper medical care and regular follow ups the child born with HLHS can now live a full and close to normal and active life!  

Our family is quite familiar with this condition as my granddaughter was born with HLHS.  She is now a happy healthy energetic 9-year-old with a strength and zest for life that is inspiring to us all! We continue to value the work of the cardiology team at Norton’s Children’s Hospital in Louisville, KY as they performed her life-giving surgeries!! AvKARE is proud to be a provider of pharmaceuticals to Norton’s as well as many other facilities throughout the United States.