Late complications associated with the Fontan circulation

Abstract

The recent significant reduction in operative mortality associated with surgical completion of the Fontan circulation is clearly multi-factorial. Better understanding of the fundamental physiological and anatomical issues have led to refinements in operative technique, early neutralization of potential risk factors, widespread use of a staged approach, and selective application of the concept of fenestration or incomplete partitioning. While further reduction of operative mortality is important, and may be anticipated in the future, major emphasis now must be placed on optimizing functional outcome, and understanding and managing the late complications associated with the unique physiology produced by the Fontan circulation.