Exercise invasive hemodynamics in adults post-Fontan: A novel tool in understanding functional limitation and liver disease.

Functional impairment is essentially universal among adults post-Fontan palliation. Cardiac catheterization is frequently performed in patients post-Fontan but normal resting Fontan and ventricular filling pressures are commonly recorded even in highly symptomatic individuals. Ascertaining the etiology of exertional symptoms in these individuals is further complicated by the prevalence of co-existent sarcopenia, abnormal skeletal muscle function, impaired respiratory mechanics, and deconditioning. Exercise right heart catheterization is increasingly used in acquired heart disease but the diagnostic yield of exercise at the time of cardiac catheterization in Fontan patients remains largely unknown. We report herein exercise catheterization findings in 4 consecutive adults post-Fontan referred to our laboratory. We believe this novel series illustrates several potential roles for exercise during catheterization of adults post-Fontan, with each patient having a unique constellation of hemodynamic findings to explain their clinical presentation and tailor their management.