Since 1970, when the first nationwide survey of Kawsaki disease was conducted, a total of 21 surveys have been conducted every 2 years in Japan; the latest one (the 21st) was in 2011. After the 3 nationwide epidemics in 1979, 1982, and 1986 there has no such nationwide epidemic, but the number of patients and incidence rate increased gradually since the mid-1990's. Thus the incidence rate in 2010 was higher than that in 1982. The etiology is still unknown, but epidemiologic data, such that the number of patients was the highest in January and a small elevation was observed in summer, and that age-specific incidence rate was monomodal where the peak existed at 6-11 months of age, indicate that some infectious agents might affect the onset of the disease. On the other hand, parent-child cases and sibling cases indicate a host factor. The prevalence of cardiac lesions in the acute phase and cardiac sequelae one month after the onset decreased steadily. Besides, we summarized the results of the analyses for the nationwide survey data in detail and analyzede the data in addition to other sources in this review article.

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Despite recent progress in surgical outcomes for congenital malformations in neonates and young infants, postoperative myocardial dysfunction due to ischemic-reperfusion injury remains a major contributor to morbidity and mortality in the settings of increasingly complex surgical procedures and longer ischemic duration.
A wide variety of clinical profiles of pediatric patients in addition to a number of conflicting experimental results concerning the role of cardioplegia in immature hearts may preclude a definitive conclusion to determine the most favorable cardioplegic strategy in the field of pediatric myocardial protection.
Recently, randomized comparative clinical trials were conducted to compare crystalloid and blood cardioplegia (BCP) for pediatric heart surgery, and the majority of studies demonstrated superior cardioprotective effects of BCP coupled with terminal warm BCP in terms of a reduction in biochemical injury, including myocardial ATP content and cardiac troponin release, at least in the selected categories of patients (i.e., prolonged aortic clamping and cyanotic patients). Nevertheless, the multi-institutional randomized study with an appropriate stratification according to the patient age, ischemic time, and type of procedure will be mandatory in order to conclude the true role of individual cardioplegic solution. In view of suboptimal myocardial protection by the standard cardioplegic solution of immature hearts, which are associated with the unique structural and functional characteristics and often exacerbated by preoperative hypoxia and pressure and volume overload, a recent trend in investigations has been oriented towards the development of the cardioplegic solution specialized for pediatric hearts (i.e., del Nido solution containing lidocaine and magnesium). Furthermore, a novel concept of myocardial protection, including normokalemic polarizing arrest with adenosine and lidocaine to substitute for the standard depolarizing cardioplegia and ischemic pre/post conditioning as a supplementary therapeutic modality has been tested clinically in pediatric open-heart surgery.

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Interventional catheterization for congenital heart diseases, such as balloon dilation for stenotic valves or vessels, stenting, transcatheter closure of persistent arterial ducts and atrial septal defects, is now an indispensable treatment option. However, device lag in our country constitutes a serious obstacle for introduction of several devices, such as the radiofrequency guidewire, covered stent, percutaneous pulmonary valve, and so on, which will contribute to improved prognosis of patients with congenital heart diseases treated with less invasive procedures. In this review, I would like to introduce several novel devices that are expected to be approved in our country in the near future.

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Background: A single infusion of intravenous immunoglobulin (IVIG) in a dose of 2 g per kg of body weight is the recommended treatment for Kawasaki disease (KD). However, physicians sometimes hesitate to administer this dose of IVIG to patients with heavy body weight. This study aimed to investigate the characteristics, treatments, and outcomes of KD patients withe heavy body weight.
Patients and Methods: Thirteen KD patients above 25 kg of body weight (25KD) were compared with 326 below 15 kg (15KD) regarding laboratory findings, treatments, and outcomes.
Results: The mean age and body weight of the 25KD patients were 8 ± 2 years and 30 ± 6 kg, respectively. The 25KD patients had a significantly higher neutrophil proportion and C-reactive protein levels compared with 15KD patients. They also had significantly lower mean platelet counts and a higher mean Gunma score. IVIG treatment was initiated in twelve 25KD patients within their seventh illness day. The proportion of the recommended IVIG dose (g/day) administered to the 25KD patients averaged 92% for each IVIG regimen, which was significantly less than that of the 15KD patients (112%). The difference in the incidence of coronary artery abnormality between 25KD and 15KD patients was not statistically significant.
Conclusions: The severity of the disease was worse in the 25KD patients compared with 15KD patients. However, following the initial treatment with IVIG, no 25KD patients required an additional IVIG treatment in this study. Further investigation to determine the adequate IVIG dose for KD patients withe heavy body weight is necessary.

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Background: There was a limitation of Perioperative transesophageal echocardiography (PTEE) in small infants.
Purpose: We examined the effectiveness of PTEE with a pediatric multiplane probe (PMP).
Methods: Children who underwent PTEE (n=412) were divided according to probe type. PTEE was done with a PMP in Group A patients, with a pediatric biplane probe in Group B, with an adult multiplane probe in Group C, and PTEE was unsuccessful in Group D. Age distribution, basic cardiac disease, additional surgical procedures, postoperative course, and reasons for and effects of unsuccessful probe insertion were investigated.
Results: In Group A, 86% of the subjects were 0-4 years old (<1 year, 33%), 57% in Group B were 5-9 years old, 70% in Group C were 15 years or older, and 83% in Group D were less than 1 year old. An additional surgical procedure was undergone in 16 in Group A and 6 in Group C. Regarding cardiac disease, a simple heart defect was found in 35% in Group A, 94% in Group B, and 48% in Group C. In Group A, right ventricular outflow tract reconstruction was performed in seven patients, atrioventricular valve repair in 6, residual shunt closure in 3, and stenosis release of the superior vena cava in 1. In Group D, extra-cardiac anomalies and low body weight were frequent findings, and 1 required a reoperation on postoperative day 15.
Conclusion: PTEE using a PMP was highly beneficial for cardiac surgery in small children with complex cardiac anomalies, leading to improved prognosis.

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Protein-losing enteropathy (PLE) is one of the major complications after Fontan operation and usually results in a poor outcome. The mechanism of PLE is not fully understood, and the specific treatment remains to be studied. Recently, some reports showed that the phosphodiesterase inhibitor V, sildenafil, might improve PLE through the vasodilation of not only the pulmonary artery but also the mesenteric artery. Here, we report three patients with PLE after the Fontan operation whose symptoms were improved by administration of sildenafil. [Case 1] A twenty-one-year old male with single ventricle developed edema due to PLE 6 years after surgery. Sildenafil was administered at a dose of 30 mg per day, and edema was resolved after titrating it up to 40 mg per day. [Case 2] A seventeen-year-old male with single ventricle developed cyanosis and edema due to pulmonary arterial-venous fistula and PLE 2 years after surgery. Sildenafil was administered at a dose of 1 mg/kg per day, and his symptom was resolved after titrating it up to 4 mg/kg per day. [Case 3] A twelve-year-old girl with double outlet right ventricle developed edema and ascites due to PLE a year after surgery. The symptoms were refractory to oral prednisolone (1 mg/kg per day). After sildenafil was administered at a dose of 0.5 mg/kg per day and titrated up to 8 mg/kg per day, ascites finally resolved. No major complications were observed in each case. Sildenafil may be a safe and effective therapeutic option for PLE after the Fontan operation with dose dependent efficacy.

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