Treatment of hypoplastic left heart syndrome (HLHS) typically requires 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, known as the Norwood procedure, occurs within a few days of birth. The illustration above shows a “Blalock-Taussig” shunt. Alternative types of shunts may be used based upon a child’s individual anatomy.
Stage II, known as the bidirectional Glenn, typically occurs within four to six months of birth. An alternative technical modification called the “Hemi Fontan” (not shown) may be used based on a child’s individual anatomy.
Stage III, known as the Fontan procedure, typically occurs between one-and-a-half to four years of age. The illustration above shows a technique called an “extracardiac Fontan”. In some children a different modification, termed a “lateral tunnel fenestrated Fontan”, is utilized.
The approach above is the most common for HLHS, however, alternative approaches may be recommended for some children, including transplantation or “hybrid” procedures. An individualized approach is taken for each and every child. Your doctor will explain each option and what approach might be recommended for your child.