The ‘failing’ Fontan circulation

While functional outcomes for Fontan patients continue to improve, increasing follow-up underscores the impact of the single ventricle circulation on all body tissues and organ systems. In long-term follow-up, a variety of potential systemic effects and complications may develop. Optimization of quality and quantity of life for these patients requires an awareness for, and understanding of, the spectrum of these complications. Medical, interventional and surgical therapies continue to evolve although further investigation is required to determine the utility of many of these in this particular population.