What is the final stage of surgical repair for hypoplastic left heart syndrome?

The final stage of surgical repair for hypoplastic left heart syndrome is the Full Fontan procedure. This complex condition necessitates a series of surgeries that enable proper circulation in children who are born with an underdeveloped left side of the heart.

The initial phase of treatment typically involves the Norwood procedure, which establishes a new systemic flow to the body. This is followed by either the Bidirectional Glenn or Hemi-Fontan procedures, which help to direct blood flow from the upper body to the lungs. However, these are not the final stage.

The Full Fontan procedure is performed later in life, usually between 18 months and 4 years of age, and it completes the surgical palliation for hypoplastic left heart syndrome. At this stage, the remaining circulation is redirected so that blood returning from the body is routed directly to the pulmonary arteries, effectively separating the systemic and pulmonary blood flow, improving oxygenation and overall function.

This final stage is critical as it significantly enhances the quality of life and outcomes for patients by eliminating any mixing of oxygenated and deoxygenated blood and maintaining a single ventricle's workload.