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Anticoagulant regimens in Fontan patients varied widely with a significant trend for warfarin use in patients with impaired haemodynamics. Low arterial oxygenation may predict haemostatic events. The relatively high prevalence of haemorrhagic complications indicates the need for individualized anticoagulant administration throughout the follow-up.

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 systemic 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.

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