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.

No abstract

No abstract

An 11-year-old boy was admitted to our hospital with a 4-day history of severe sore throat and a 2-day history of fever and cervical lymphadenopathy. A rapid antigen test for beta-hemolytic streptococci, performed at another hospital, was negative. He had varicella at 5 months of age; he was not administered a varicella vaccine afterwards.On admission, his vital signs were as follows: temperature, 37.3°C; pulse, 87 beats/min; and percutaneous oxygen saturation 98%. He complained of severe sore throat and odynophagia. Physical examination revealed a reddish throat and bilateral cervical lymphadenopathy. Rashes were not observed on his head, neck, trunk, nor his extremities. Because his sore throat was so severe, contrast-enhanced computed tomography was performed. The examination revealed thickening and enhancement of the left side of the pharyngolaryngeal mucosa a b c d Fig. 1 (a) Appearance of umbilical granuloma at 14 days of age. (b, c) Abdominal Doppler ultrasonography finding of an umbilical arteriovenous fistula. (b) Arterial blood was supplied from the abdominal wall. (c) The umbilical arterial blood drained into the umbilical vein. (d) The umbilicus after reconstruction.