Half a century has passed since the beginning of school-based electrocardiographic cardiovascular (CV) screening in Japan. This screening in children and adolescents who have life-threatening CV diseases might be able to prevent sudden cardiac death. Our knowledge has grown and many premises of pediatric cardiology have been renewed by findings from this screening. Only Japan has established a nation-wide CV screening system. However, these facts are not known outside of Japan. This implies that we (members of the Japanese Society of Pediatric Cardiology and Cardiac Surgery) have not provided sufficient information regarding the academic, social, and cost-effectiveness of this system. The author considers that this screening system needs to be improved. We need to continuously provide information concerning data from the electrocardiographic CV screening system.

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Purpose: We evaluated the early and mid-term results of Fontan operation in adults.
Methods: Since January 1998 until July 2012，a total of 6 patients (3 male and 3 female) totally underwent Fontan operation at our institution. The age was 22 ± 5 years (range 17-31). Operation methods，pre and postoperative catheterizationresult，New York Heart Association classification (NYHA)，cardio-thoracic ratio，BNP and liver function were retrospectively reviewed from patients’ medical records.
Result: There were no hospital deaths. All patients have been doing well. Four patients underwent primary Fontan surgery, and other 2 patients underwent staged bidirectional Glenn surgery. We performed 5 extracardiac conduit Fontan procedures and 1 intra cardiac conduit procedure without fenestration. Preoperative mean pulmonary artery pressure，pulmonary arterial resistance，and pulmonary arterial index were 12 ± 3mmHg，1.05 ± 0.61U/m²，and 443 ± 152, respectively. In all patients, the cardiac function was well preserved，no atrio-ventricular valve regurgitation was seen, and NYHA class improved from II to I. There was no remarkable change in cardio-thoracic ratio，BNP or liver function during 10 years of follow-up after the operation.
Conclusion: Our results indicate that adults can safely undergo the Fontan operation the same as children. However，careful follow-up is necessary since the long-term results are unknown.

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Background: In children, we need to decide the indications for implantable cardioverter defibrillator (ICD) implantation in consideration of somatic growth potential, complications, and long-term maintenance. In Japan, few studies on ICD therapy have been reported.
Objective: This study was performed to examine the indications for ICD and situations after ICD implantation.
Method: Six patients with first ICD implantation at <18 years old were examined. Demographic information, indications, approach, appropriate and inappropriate discharge data, additional therapy (catheter ablation and medication) and complications were recorded for all patients.
Results: The underlying pathology was idiopathic ventricular fibrillation (Vf) in two patients, and ventricular tachycardia after repaired tetralogy of Fallot, ventricular tachycardia after fulminant myocarditis, dilated cardiomyopathy, and long QT syndrome in one patient each. According to the guidelines of the Japanese Circulation Society, the criteria for ICD implantation were judged as class I in five patients and unclassified in one patient. The median age at ICD implantation was 12.9 years (range, 3.8-17.9 years). Duration of follow-up was 3.0 years (range, 0.8-4.9 years). Three patients received four appropriate discharges. One patient received four inappropriate discharges. Catheter ablation was performed in two cases, and medication for preventing arrhythmia was administered in five cases. No major complications were encountered during follow-up.
Conclusion: Indications for ICD therapy appear appropriate because most patients with class I experienced significant rates of appropriate discharge. No major complications were seen during follow-up. Even in the pediatric population, ICD implantation for class I patients is beneficial for preventing sudden cardiac death.

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Background and Methods: To delineate the clinical characteristics of brain abscess concomitant with cyanotic congenital heart disease in Japan, a nationwide survey was performed through the network of the Committee for Genetics and Epidemiology of Cardiovascular Diseases in the Japanese Society of Pediatric Cardiology and Cardiac Surgery.
Results: Of ninety reported cases, 75 cases (83％) survived, 6 cases (7％) died, and the remaining 9 had unknown outcome. Forty-five of 83 cases (54％) showed neurologic symptoms such as loss of consciousness, seizure, and palsy. Seventeen of 90 cases (19％) had evidence of preceding symptoms including upper respiratory or gastrointestinal infection. Although dental procedures were performed ahead of brain abscess in eight cases (9％), seven of these cases (88％) were not given prophylactic antibiotic treatment against infective endocarditis. Bacterial organism were identified in the blood or abscess culture in 32 of 42 examined cases (76％) and Streptococcus species were the most frequently found (53％). The level of serum C-reactive protein was less than 1mg/dL in 72％ of patients at the diagnosis. Neurologic sequelae remained in 31 of 75 survived cases (41％) and were significantly common in those who manifested neurologic symptom at the diagnosis (p<0.01).
Conclusion: Lower level of inflammatory response in blood examination was common in patients with brain abscess concomitant with cyanotic congenital heart disease. The antimicrobial prophylaxis of infective endocarditis for dental procedures was suggested to be important to prevent brain abscess. Focal neurologic symptoms at the onset of brain abscess were significantly associated with neurologic sequelae thereafter.

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This report describes the efficacy of tolvaptan for the treatment of protein-losing enteropathy (PLE) that developed in two patients who underwent the Fontan operation. The first patient was an 8-year-old boy with asplenia, dextrocardia, common atrioventricular canal (CAVC), univentricular heart (UVH), pulmonary atresia and total anomalous pulmonary venous connection. Although intravenous heparin, oral corticosteroids and intravenous albumin were administered, serum albumin and total protein levels were only approximately 2 g/dL and 3 g/dL, respectively. Subsequent administration of tolvaptan resulted in normalization of serum albumin and total protein levels and a decrease in PLE-related diarrhea. The second patient was a 19-year-old man with polysplenia, UVH, CAVC, double-outlet right ventricle, pulmonary atresia and interrupted inferior vena cava (azygos connection). Tolvaptan administration in this patient resulted in an increase in serum albumin and total protein levels and a decrease in ascites. These observations suggest that tolvaptan may be an effective treatment option for PLE in the patients who have undergone the Fontan operation.

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A combination of completely unroofed coronary sinus and persistent left superior vena cava (PLSVC) is termed LSVC draining into left atrium (LA). LSVC draining into LA in patients with situs solitus is rare. We recently performed total correction in a 5-year-old girl with situs solitus who was diagnosed with a partial atrioventricular septal defect, PLSVC, and an abnormal septum in LA, like cor triatriatum, shortly after birth. Because she presented with mild cyanosis without heart failure, she was followed up without medication on an outpatient basis. However, left atrioventricular valve regurgitation (LAVVR) increased as detected by echocardiography, and she was admitted for thorough examination. Multidetector computed tomography (MDCT) and cardiac catheterization revealed grade Ⅱ LAVVR, LSVC draining into LA, and abnormal septum in LA. Intracardiac repair, followed by extracardiac rerouting from LSVC to the right atrium using autologous tissue was performed in addition to resection of the abnormal septum.

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Background: Although Dexmedetomidine (DEX) is primarily classified as a sedative, several foreign studies have suggested that DEX is effective for perioperative atrial and junctional ectopic tachycardia．We report the efficacy of DEX for perioperative ectopic atrial tachycardia in two infants with congenital heart disease．
Case1: Patch closure of ventricular septal defects (VSDs) using the Sandwich method and pulmonary artery (PA) plasty were carried out in an 1-year-6 month old boy who previously underwent PA banding for multiple muscular VSDs．Ectopic atrial tachycardia (EAT) with a rate of 180 bpm was initiated followed by hypotension on post-operative day (POD) 22．Overdrive pacing, amiodarone, landiolol hydrochloride (LAN), a few sedatives, as well as a muscle relaxant agent were not effective for this EAT．We discontinued LAN following hypotension complicated by left ventricular (LV) dysfunction, while started DEX infusion of 0.7μg/kg/hr．After DEX administration, EAT was terminated without hypotension．
Case2: A 2-year-6-month old boy of Truncus arteriosus, who previously underwent PA resection and rt-m-Blalock-Taussig shunt, was operated using the Rastelli procedure and VSD patch closure．EAT about a rate of 190 bpm was initiated with hypotension, -on 1POD．Overdrive pacing, a sedative, and as a muscle relaxant agent were not effective for this EAT．We started DEX infusion of 0.6μg/kg/hr．After DEX administration, EAT was terminated successfully. None of the patients developed any adverse events, such as bradycardia and hypotension．
Conclusion: DEX appears to have novel antiarrhythmic properties for acute termination of EAT on perioperative infants associated with congenital heart disease．This report suggests that DEX may safely terminate EAT in cases complicated by LV dysfunction．

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