Hypoplastic Left Heart Syndrome: After Diagnosis

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Nov 12, 2014 | Suzanne Ferguson | @suzannerferguson

After your baby has been diagnosed with HLHS, doctors will determine the exact treatment for your baby depending on the severity of your baby's heart condition and your family’s wishes. HLHS requires aggressive, early surgical intervention. The most common approach involves a three-stage surgical procedure. The operations are performed within the first few years of life and create reliable blood flow in and out of the functional heart chambers.

Stage 1: Norwood Procedure The Norwood procedure is performed within the first few days of life to allow the heart’s lower right chamber, known as the right ventricle, to pump blood to the lungs and the body.  

Stage 2: Glenn Procedure The Glenn (or hemi-Fontan) procedure usually takes place when your child is between four and six months of age. The surgery reduces the amount of work the right side of the heart does by allowing it to pump blood mainly to the body and routing part of the blood returning from the body directly into the lungs.  

Stage 3: Fontan Procedure The Fontan procedure is performed when your child is between 18 and 48 months old to connect the remaining blood vessels carrying blood from the body to the lungs. The purpose of this surgery is to allow the blood coming from the body to go directly to the lungs, normalize the oxygen levels, and reduce the right ventricle workload to the approximate levels of a normal heart.  

Children with HLHS need long-term surveillance of the reconstructed heart and often require heart medications. Regular appointments with a cardiologist will include echocardiograms, MRIs, blood tests, and heart catheterizations to ensure close monitoring of your child’s growing heart.

The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of delaying or preventing heart failure for individuals affected by congenital heart defects including HLHS. The specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science, to diagnostic imaging to regenerative therapies.