Recently, it is common to perform the Fontan procedure after the Glenn procedure as surgical repair for the univentricular heart. How the brain oxygen saturation (rSO) values change with the cardiac restoration and the process of growth during these procedures in individual children remains unknown. In this study, we retrospectively studied rSO data as well as the perioperative clinical records of 30 children who underwent both Glenn and Fontan procedures by the same surgeon in the same institute. The rSO was measured at the beginning and end of each procedure with an INVOS 5100C. Cerebral perfusion pressure was calculated by subtracting central venous pressure from mean arterial pressure. Arterial oxygen saturation (SaO) and the hemoglobin concentration were obtained as candidates affecting rSO changes at the start and the end of both procedures. The rSO increased during the Glenn procedure, but this increase was slight and insignificant. On the other hand, the rSO significantly increased during the Fontan procedure. Significant increases in SaO were observed only between the beginning and end of the Fontan procedure. Correlation coefficients determined by linear regression analysis were more than 0.5 between rSO and SaO in both procedures. Multiple linear regression analysis showed that SaO was the key determinant of the rSO. The rSO increases step by step from the Glenn to the Fontan procedure in the same patient. Within each procedure, SaO is the key determinant of the rSO. The significance of rSO monitoring in these procedures should be further evaluated.

Background Patients with right isomerism have accompanying complex congenital heart disease, which is characterized by pulmonary atresia and total anomalous pulmonary venous return. Balanced regulation of the systemic and pulmonary circulation is essential for successful management, especially for cases complicated with necrotizing enterocolitis (NEC). Case presentation A 6-day-old male neonate with a single ventricle, pulmonary atresia, patent ductus arteriosus (DA), and total anomalous pulmonary venous return associated with right isomerism was admitted because of dyspnea, cyanosis, and melena. The patient presented circulatory incompetence due to excessive pulmonary blood flow, resulting in NEC. The patient underwent DA banding and colectomy following continuous intravenous infusion of prostaglandin E1 at six days. Subsequently, his condition improved, reaching a systemic oxygen saturation of around 80%. He underwent a bidirectional Glenn procedure and closure of colectomy at the ages of 5 and 6 months, respectively. Conclusion DA banding can be an alternative to placing an aortopulmonary shunt, which is conventional in patients with ductus-dependent pulmonary circulation, because DA banding is feasible without cardiopulmonary bypass.

Background: Diabetic ketoacidosis (DKA) and hyperglycemic hyperosmolar syndrome (HHS) are life-threatening complications of diabetes mellitus. Their clinical profiles have not been fully investigated in the Japanese population. Methods: A multicenter retrospective cohort study was conducted in 21 acute care hospitals in Japan. Patients included were adults aged 18 or older who had been hospitalized from January 1, 2012, to December 31, 2016 due to DKA or HHS. The clinical characteristics and outcomes were extracted from patient medical records. A four-group comparison (mild DKA, moderate DKA, severe DKA, and HHS) was performed to evaluate outcomes. Results: A total of 771 patients including 545 patients with DKA and 226 patients with HHS were identified during the study period. The major precipitating factors of disease episodes were poor medication compliance, infectious diseases, and excessive drinking of sugar-sweetened beverages. The median hospital stay was 16 days [IQR 10-26 days] and was longer in the HHS group (19.5 days) compared to the DKA groups (16 days). The intensive care unit (ICU) admission rate was 44.4% (mean) and the rate at each hospital ranged from 0% to 100%. The median ICU stay was 3 days for all groups. The in-hospital mortality rate was 2.8% in patients with DKA and 7.1% in the HHS group. No significant difference in mortality was seen among the three DKA groups. The most common complication was infection (18%), followed by pulmonary edema (2.7%), stroke (2.1%), ventricular arrhythmia (1.6%), and deep vein thrombosis (1%). Conclusions: The mortality rate of patients with DKA in Japan is similar to other studies, while that of HHS was lower. The ICU admission rate varied among institutions. There was no significant association between the severity of DKA and mortality in the study population. Trial registration: This study is registered in the UMIN clinical trial registration system (UMIN000025393, Registered 23th December 2016)

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