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In the late phase after Fontan surgery, organ dysfunction due to high central venous pressure (CVP) is a major clinical problem. We have described the cases of two patients with portosystemic shunts who exhibited hyperammonemia and high cardiac output associated with peripheral vasodilatation after Fontan surgery. A high CVP in these patients may have resulted in the formation of a portosystemic shunt. We performed coil embolization and balloon-occluded retrograde transvenous obliteration for each case. The possibility of a portosystemic shunt as a postoperative complication of Fontan surgery should always be considered. Early detection and therapeutic intervention seem necessary from the viewpoint of stabilizing the Fontan circulation and delaying the progression of liver disorder.

Introduction The short-term survival rate after single-stage correction of Taussig-Bing anomaly with aortic arch obstruction remains favorable. However, some cases are encountered occasionally in which single-stage correction was not performed during the neonatal period. Accordingly, we evaluated the midterm outcomes of different surgical strategies. Methods Seven patients who underwent an arterial switch operation and intraventricular rerouting as definitive surgery between 2007 and 2017 were investigated. Of these 7 patients, 3 had undergone previous pulmonary artery banding and aortic arch reconstruction. Results The median body weight at definitive surgery was 3.3 kg (range 2.9–8.3 kg). At definitive surgery, the arrest time for single-stage correction (162.3 ± 21.7 min) was significantly shorter than that of staged repair (206.3 ± 5.1 min, p = 0.020). There was no hospital or late death. One patient in both strategy groups underwent aortic reintervention 54 months and 7.1 months after the definitive operation. Neoaortic valve (perinatal pulmonary valve) diameter decreased significantly from the perinatal valve diameter following definitive surgery (median +4.94z and +2.12z, respectively, p = 0.016) but there was no significant difference in the neopulmonary valve (perinatal aortic valve) diameter. Both single-stage correction and staged repair patients showed a similar trend. At the last follow-up, no patient had greater than mild neoaortic or neopulmonary valve regurgitation. Conclusion The surgical outcomes of both single-stage correction and staged correction for Taussig-Bing anomaly with aortic arch obstruction are excellent. Both strategies produce similar changes in the diameter and regurgitation grade of the neoaortic and neopulmonary valves.

OBJECTIVES Bilateral pulmonary artery banding (bPAB) is utilized for some patients with a ventricular septal defect (VSD) and aortic coarctation (CoA) or interrupted aortic arch (IAA). We evaluated aortic valve (AoV) diameter and patient outcomes following bPAB. METHODS Between August 2010 and September 2015, 10 consecutive patients with VSD and patent ductus arteriosus-dependent CoA or IAA underwent bPAB because of an AoV diameter of approximately

Summary Background Genetic studies have indicated possible involvement of the upregulated calcium-nuclear factor of activated T cells pathway in the pathogenesis of Kawasaki disease. We aimed to assess safety and efficacy of ciclosporin, an immunosuppressant targeting this pathway, for protection of patients with Kawasaki disease against coronary artery abnormalities. Methods We did a randomised, open-label, blinded endpoints trial involving 22 hospitals in Japan between May 29, 2014, and Dec 27, 2016. Eligible patients predicted to be at higher risk for intravenous immunoglobulin (IVIG) resistance were randomly assigned to IVIG plus ciclosporin (5 mg/kg per day for 5 days; study treatment) or IVIG (conventional treatment) groups, stratified by risk score, age, and sex. The primary endpoint was incidence of coronary artery abnormalities using Japanese criteria during the 12-week trial, assessed in participants who received at least one dose of study drug and who visited the study institution at least once during treatment. This trial is registered to Center for Clinical Trials, Japan Medical Association, number JMA-IIA00174. Findings We enrolled 175 participants. One patient withdrew consent after enrolment and was excluded and one patient (in the study treatment group) was excluded from analysis because of lost echocardiography data. Incidence of coronary artery abnormalities was lower in the study treatment group than in the conventional treatment group (12 [14%] of 86 patients vs 27 [31%] of 87 patients; risk ratio 0·46; 95% CI 0·25–0·86; p=0·010). No difference was found in the incidence of adverse events between the groups (9% vs 7%; p=0·78). Interpretation Combined primary therapy with IVIG and ciclosporin was safe and effective for favourable coronary artery outcomes in Kawasaki disease patients who were predicted to be unresponsive to IVIG. Funding Japan Agency for Medical Research and Development (grant CCT-B-2503).

Sporadic congenital none-autoimmune hyperthyroidism is rare disease. It causes the gain of function with TSH receptor gene mutation. We report 3 months old Japanese boy with sporadic congenital none-autoimmune hyperthyroidism. Our case was born in the 34th week of gestation with a birth weight 1830g, his length 42.0cm, his head circumference 29.5 cm as low birth weight baby between non consanguineous parents. Oligohydramnios was pointed out his perinatal period. There was no history of thyroid disease in other family members. He showed failure to thrive after the age of one month in spite of increasing nutrition. At the age of 3 months, His weight was 3.5kg, his length 54.6cm. He presented with tachycardia (170~180/min), severe sweating, mild exophthalmos and no goiter. We examined carefully and found he was suffered from hyperthyroidism. The thyroid function showed TSH A,p.Ser281Asn that had been reported before. The boy was treated with amount of 0.8 mg/kg/day of methimazole, his irritability disappears and his heart rate is down. Now his weight gains slowly and his development is appropriate for his age. We conclude that careful examination and follow up need for his development and growth because the severity of sporadic congenital none-autoimmune hyperthyroidism is variety and most reported case were recurrent. Written informed consent was obtained from the patient's parent or guardian for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.