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We had a case of Listeria monocytogenes (LM) meningitis complicated with hypercytokinemia and hemophagocytic lymphohistiocytosis in a healthy 22-month-old boy. He was admitted to our hospital with a fever, vomiting, mild consciousness disturbances, and extraocular muscle paralysis. Magnetic resonance imaging (MRI) revealed bilateral deep white matter lesions. After receiving ampicillin, meropenem, and gentamicin, his cerebrospinal fluid (CSF) culture results turned negative on the third day of hospitalization. However, the fever intermittently persisted, and it took approximately 40 days to completely resolve. During this period, various inflammatory cytokine levels, particularly neopterin, in the blood and CSF remained elevated. Therefore, long-term administration of corticosteroids in addition to antibiotics was required. The use of dexamethasone appeared to be effective for neurological disorders such as consciousness disturbance and extraocular muscle paralysis associated with abnormal brain MRI findings. LM meningitis may present with encephalopathy and persistent fever due to hypercytokinemia. In such cases, corticosteroid therapy should be considered. [ABSTRACT FROM AUTHOR]

A 33-year-old man, who had undergone atriopulmonary connection Fontan procedure (AP Fontan) for double outlet right ventricle, suffered from heart failure due to atrial tachycardia at 27 years old. Atrial tachycardia was suppressed after amiodarone administration. At 32 years old, atrial tachycardia recurred, and short palpitations gradually increased. Cardiac computed tomography showed that coronary sinus (CS) was perfused into the pulmonary venous atrium, and catheter insertion to CS from the systemic venous atrium was impossible. We performed an electrophysiology study (EPS) and radiofrequency catheter ablation (RFCA) under local anesthesia. An esophageal electrode catheter was inserted as a potential reference for ultra-high density three-dimensional (3D) mapping system. Two types of atrial tachycardia were induced by EPS. Ultra-high-density 3D mapping system revealed an intra-atrial reentrant pattern around the scar area on the lower right atrium in both atrial tachycardias; therefore, we diagnosed intra-atrial reentrant tachycardia (IART). The low voltage area and inferior vena cava during IART were ablated linearly, and IART was terminated. In conclusion, a CS electrode catheter cannot be inserted in a patient with AP Fontan, and ultra-high-density 3D mapping using the esophageal electrode catheter as a potential reference enables accurate and rapid mapping and is very effective for RFCA. The incidence of intra-atrial reentrant tachycardia (IART) is high in patients with late after Fontan procedure, and the treatment may be difficult. Ultra-high-density three-dimensional mapping can perform accurate mapping of IART and rapid detection of low voltage areas effective for radiofrequency catheter ablation. An esophageal electrode catheter can be the reference potential for accurate activation mapping in Fontan patients where coronary sinus electrode catheter insertion may be impossible from a systemic venous atrium. [ABSTRACT FROM AUTHOR]

A 9-year-old boy, diagnosed with double outlet right ventricle after birth, suffered sinus node dysfunction and non-sustained junctional tachycardia after an extracardiac total cavopulmonary connection (TCPC). Spontaneous atrial tachycardia appeared 3 years after an extracardiac TCPC. Sotalol was administered but the bradycardia was obvious. It was difficult to increase sotalol and atrial tachycardia was uncontrollable. Atrial tachycardia continued with symptoms; direct current (DC) cardioversion was frequently required. Five years after extracardiac TCPC, we implanted a pacemaker with atrial antitachycardia pacing (ATP) using epicardial leads. On day 2 post operation, wide QRS tachycardia appeared. Due to decreased blood pressure, DC cardioversion was immediately performed, but it recurred from atrial premature contraction. We judged this was atrial tachycardia with 1:1 atrioventricular conduction based on an intracardiac electrogram and it was terminated by burst atrial pacing from the pacemaker. After changing atrial pacing rate to 150 ppm, atrial tachycardia could be suppressed. Due to atrial pacing and increasing sotalol gradually, junctional tachycardia terminated spontaneously, and atrial tachycardia was not induced after pacemaker implantation. In conclusion, implantation of a pacemaker with ATP and intensification of antiarrhythmic drugs is an effective treatment strategy for pediatric patients with bradycardia-tachycardia syndrome after extracardiac TCPC. < Learning objective: The treatment for bradycardia-tachycardia syndrome in children after extracardiac total cavopulmonary connection (TCPC) is challenging. The appropriate antiarrhythmic drugs for atrial tachycardia cannot be administered due to bradycardia, and it is often difficult to perform radiofrequency catheter ablation on small children. Surgical pacemaker implantation, although invasive, is the most effective treatment for bradycardia-tachycardia syndrome in small children after extracardiac TCPC.> [ABSTRACT FROM AUTHOR]

Objective: To determine the utility of capillary blood ketone levels as an indicator of inadequate intake of breast milk in the early postnatal period.Study Design: Levels of capillary blood beta-hydroxybutyrate (βOHB), the main ketone body in the blood, were measured with a bedside ketone meter in 585 full-term neonates aged 48-95 hours who were breastfed exclusively. Relationships between weight-loss percentage, blood sodium, glucose, pH, partial pressure of carbon dioxide, base-deficit levels, and βOHB levels were investigated. The diagnostic accuracy of βOHB for predicting excessive weight loss (weight loss ≥10% of birth weight) and hypernatremic dehydration (blood sodium level ≥150 mEq/L) was determined.Results: βOHB levels were correlated positively with weight-loss percentage and blood sodium levels and were correlated negatively with blood glucose levels. The diagnostic accuracy of βOHB was 0.846 (optimal cut off, 1.55 mmol/L; sensitivity, 80.9%, specificity, 74.0%) for predicting excessive weight loss and 0.868 (optimal cut off, 1.85 mmol/L; sensitivity, 94.3%; specificity, 69.9%) for predicting hypernatremic dehydration according to the area under the receiver operating characteristic curve. Multiple logistic analysis revealed that βOHB and weight loss percentage were the only independent predictors of hypernatremic dehydration. Increases in βOHB levels also were associated with worsening metabolic acidosis and hypocapnia.Conclusion: High βOHB levels were associated with inadequate intake of breast milk in the early postnatal period. The use of bedside capillary blood ketone levels may be clinically useful as an indicator of dehydration, energy depletion, and acid-base imbalance in breastfeeding infants in the early postnatal period. [ABSTRACT FROM AUTHOR]

We report the case of a 13-year-old boy who, while running in a school gymnasium, experienced sudden syncope and seizure. CPR was started immediately, and an automated external defibrillator (AED) was attached, but shock was not induced. He was referred to our hospital for loss of consciousness and intermittent general tonic-clonic seizure. A 12-lead electrocardiogram showed normal sinus rhythm and no ST-T wave abnormalities. Echocardiography showed normal structural heart and normal cardiac function. On the second day of hospitalization, AED electrocardiogram showed complete atrioventricular (AV) block at syncope and seizure. After the patient recovered from this neurological state, we performed the treadmill exercise test, and it did not show ST-T wave abnormalities or AV block, and he did not complain of chest pain. Coronary angiography showed atresia of the left main trunk and the collateral vessel from the right coronary artery connected to the left coronary artery. He was diagnosed with congenital left main coronary artery atresia. We began administration of calcium antagonist and aspirin to prevent a coronary artery spasm and then performed a coronary artery bypass graft (CABG) to prevent sudden cardiac death. After CABG, he has had no syncope episodes at rest or during light exercise. < Learning objective: In pediatric patients, syncope during strenuous exercise should mandate exclusion of cardiac events, especially coronary artery anomalies. Coronary artery anomalies that could cause sudden cardiac death sometimes show no abnormalities at rest or even during exercise stress on 12-lead electrocardiogram. It is very important to suspect cardiogenic syncope during strenuous exercise.> [ABSTRACT FROM AUTHOR]

The left posterior and posteroseptal accessory pathways often have an epicardial accessory and are associated with coronary vein anomalies, such as diverticulum, fusiform, or bulbous enlargement. We report the case of a 15-year-old boy who suffered from palpitation due to Wolff–Parkinson–White syndrome with coronary sinus diverticulum. An electrophysiology study revealed a left posterior accessory pathway and orthodromic atrioventricular reciprocating tachycardia. After the transseptal puncture, we performed mapping around the mitral annulus during sinus rhythm. We could not detect typical atrioventricular fusion accompanied with accessory pathway potential and failed to ablate around the mitral annulus. We revealed typical accessory pathway potential in a coronary vein and successfully ablated. After ablation, a right atrium angiography showed a successful ablation site was just at the neck of coronary sinus diverticulum. It is important for a successful and safe ablation to evaluate coronary vein anomalies in patients with left posterior and posteroseptal accessory pathways. < Learning objective: In pediatric patients, Wolff–Parkinson–White syndrome with the posteroseptal accessory pathway with coronary sinus diverticulum is rare. It is important to predict the left posterior and posteroseptal accessory pathways from the polarity of the delta wave; ablation for pediatric patients seems to be more effective and safe in confirming coronary sinus diverticulum by echocardiogram before electrophysiology study and performing coronary vein angiography before mapping of the accessory pathway.> [ABSTRACT FROM AUTHOR]