Invasive Hemodynamic Evaluation of the Fontan Circulation: Current Day Practice and Limitations

Establishing the Fontan circulation has led to improved survival in patients born with complex congenital heart diseases. Despite early success, the long-term course of Fontan patients is complicated by multi-organ dysfunction, mainly due to a combination of low resting and blunted exercise-augmented cardiac output as well as elevated central venous (Fontan) pressure. Similarly, despite absolute hemodynamic differences compared to the normal population with biventricular circulation, the "normal" ranges of hemodynamic parameters specific to age-appropriate Fontan circulation have not been well defined. With the ever-increasing population of patients requiring Fontan correction, it is of utmost importance that an acceptable range of hemodynamics in this highly complex patient cohort is better defined.

Multiple publications have described hemodynamic limitations and potential management options in patients with Fontan circulation; however, an acceptable range of hemodynamic parameters in this patient population has not been well defined. Identification of "normal" hemodynamic parameters among patients with Fontan circulation will allow physicians to more objectively define indications for intervention, which is a necessary first step to eliminate institutional and regional heterogeneity in Fontan management and potentially improve long-term clinical outcomes.