Circulation Journal Official Journal of the Japanese Circulation Society http://www. j-circ.or.jp our decades have passed since the first Fontan operation was performed in a patient with tricuspid atresia 1 and since then a lot of patients with congenital heart disease who were not suitable for a biventricular repair have enjoyed the benefits of this procedure. The lack of a pulmonary ventricle forced us to rely on an elevated central venous pressure (CVP) and the sucking property of the

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... c ventricle to maintain the pulmonary circulation. However, these inadequate compensatory adaptations lead to a diminished preload for the systemic ventricle, resulting in a low cardiac output (CO) and, therefore, chronically elevated CVP and low CO characterize the Fontan circulation. 2 In general, we categorize Fontan patients with a low CVP and high cardiac index (CI) as "good" in terms of the hemodynamics. However, the definition of a low or high CI remains unknown. The other important clinical characteristic of the Fontan patients are a high prevalence of postoperative complications, 3 such as arrhythmias, 4 re-intervention, and non-cardiac lesions that include protein-losing enteropathy. 5-7 Thus, long-term Fontan survivors without those complications can also be categorized as those with a good clinical status. We thought that our ongoing management strategy of seeking a better long-term outcome based on serial comprehensive assessments, including cardiac catheterization and cardiopulmonary exercise testing (CPX), would enable us to clarify clinically and hemodynamically the long-term good Fontan survivors. 8 Accordingly, our purpose for the present study was to identify the long-term clinically (ie, no clinical events) and hemodynamically good Fontan survivors, to characterize the hemodynamics, and finally to determine the clinical predictors of long-term good Fontan survivors. Background: The serial hemodynamics and predictors of long-term, good Fontan survivors remain unknown.