Hypoplastic left heart syndrome (HLHS) is most often fatal without early intervention. It will typically require open heart surgery to re-direct the oxygen-rich ("red") blood and oxygen-poor ("blue") blood in a series of three reconstructive operations known as "Staged Reconstruction."
Stage I – Norwood procedure
Stage I, known as the Norwood procedure, occurs within a few days of birth. The animation to the right shows Stage 1 of reconstruction of a heart with hypoplastic left heart syndrome. The shunt that is depicted is called a “Blalock-Taussig” shunt. Alternative types of shunts may be used based upon a child’s individual anatomy.
For a small number of children, alternative approaches to the Stage I Norwood procedure may be recommended, such as heart transplantation or a combination of surgery and catheter-based treatment called a “hybrid procedure.” Compared to 25 years ago, there are now many different options for treatment of this complex heart condition; an individualized approach is taken for each and everychild. Your doctor will explain each individual option, and why one particular approach might be recommended for your child.
Stage II – Glenn procedure
Stage II, known as the bidirectional Glenn or the hemiFontan, typically occurs within four to six months of birth. The animation below shows Stage 2 of reconstruction of a heart with hypoplastic left heart syndrome.
Stage III – Fontan procedure
Stage III, known as the Fontan procedure, typically occurs between 1 1/2 to 4 years of age. The animation to the right shows Stage 3 of reconstruction of a heart with hypoplastic left heart syndrome using a technique called an “extracardiac Fontan.” The small hole intentionally placed to connect the conduit to the right atrium is called a “fenestration." In some children, a different modification, termed a “lateral tunnel fenestrated Fontan” is utilized. Your doctor will explain the differences and why one might be recommended for your child.
Frequent surveillance in infancy and early childhood is important to minimize risk factors for the eventual Fontan operation. Your child will also need a customized series of diagnostic tests between the planned stages of surgery, and throughout childhood. Additional surgical or catheter therapies, or in rare cases heart transplantation, may also be recommended.
After these operations:
- The right side of the heart will do what is usually the job of the left side - pumping oxygenated blood to the body.
- The deoxygenated blood will flow from the veins to the lungs without passing through the heart.