The Adult Patient with a Fontan

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• Fontan circulation is a palliative surgical procedure for complex single-ventricle congenital heart defects.
• Key substrates include hypoplastic left heart syndrome, tricuspid atresia, and double inlet left ventricle.
• Understanding the anatomy and physiology is crucial for managing patients post-Fontan operation.

Purpose of the Study:

• To summarize the anatomic and physiologic substrates of Fontan circulation.
• To present contemporary data on survival, morbidity, and reintervention rates.
• To outline potential therapeutic strategies for late complications.

Main Methods:

• Review of existing literature on Fontan circulation.
• Analysis of contemporary survival and morbidity data.
• Delineation of therapeutic approaches for late complications.

Main Results:

• Operative mortality is less than 1%, with 30-year survival around 89%.
• Limited exercise capacity is a common sequela, driven by reduced preload and fixed pulmonary vascular resistance.
• Potential late complications and their management are discussed.

Conclusions:

• Fontan circulation management requires understanding complex substrates and hemodynamics.
• Significant improvements in survival have been achieved.
• Ongoing research is needed to address long-term morbidity and improve quality of life.