Fontan procedure for hypoplastic left heart syndrome

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease
• Cardiovascular Surgery

Background:

• Hypoplastic left heart syndrome (HLHS) necessitates complex palliative surgical strategies.
• The Fontan operation is a key procedure in managing HLHS survivors.
• Early Fontan approaches were associated with significant mortality and morbidity, including serous effusions.

Purpose of the Study:

• To evaluate the evolution of palliative surgical techniques for HLHS.
• To compare outcomes between the initial modified Fontan operation and a subsequent staged approach.
• To assess the impact of surgical strategy on mortality and effusive complications.

Main Methods:

• Retrospective review of 354 neonates undergoing palliative reconstruction for HLHS since 1985.
• Analysis of outcomes for patients undergoing a modified Fontan operation before 1989.
• Evaluation of a staged Fontan approach implemented since 1989, including hemi-Fontan and completion procedures.

Main Results:

• The initial modified Fontan operation had 17 early and 3 late deaths among 77 patients, with 54% developing major serous effusions.
• The staged approach (hemi-Fontan followed by Fontan completion) in 121 patients resulted in 6 early and 3 late deaths, with 46% developing effusions.
• Contemporary data (since 1991) show improved survival rates: 19% mortality after initial palliation, 6% after hemi-Fontan, and 5% after Fontan completion.

Conclusions:

• A staged approach to the Fontan procedure has demonstrably improved survival rates and reduced complications in HLHS patients.
• Minimizing right ventricular volume load and optimizing ventricular function are key benefits of the staged strategy.
• Continued refinement of surgical techniques is crucial for managing complex congenital heart defects like HLHS.