Kawasaki disease (KD) is an acute multisystem vasculitic syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, KD is currently the leading cause of acquired heart diseases in children. The incidence of KD is increasing globally. The first generation of patients with KD has now reached reproductive age, and the incidence of 2-generation cases may thus increase in number. Many gene polymorphisms are associated with KD in different populations. There are many individual and racial gene variation. Susceptible gene should be identified in the KD. Individuals with certain genes predisposing to occurrence and severity of KD could be detected in different ethnic populations. This might allow special treatment or other measures to prevent the disease and possibly limit the severity and complications of KD. I reviewed and summarized Korean and international articles about gene studies. Multicenter gene studies are needed to investigate the association between specific genes and coronary artery lesions in KD.
Child ; Coronary Vessels ; Developed Countries ; Heart Diseases ; Humans ; Incidence ; Infant ; Mucocutaneous Lymph Node Syndrome*

Evidence-based medicine is a medical movement combining professional experience with the best scientific research results. Treatment of Kawasaki disease in the acute phase is directed at reducing inflammation in the coronary artery wall and preventing coronary thrombosis, whereas long-term therapy in individuals who develop coronary aneurysms is aimed at preventing myocardial ischemia or infarction. I attempt to summarize the current state of knowledge of the medical management of patients with Kawasaki disease. The recommendations are evidence-based and derived from published data wherever possible.
Coronary Aneurysm ; Coronary Thrombosis ; Coronary Vessels ; Evidence-Based Medicine ; Humans ; Infarction ; Inflammation ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia

Kawasaki disease (KD) is an acute systemic febrile illness, predominantly affecting children less than the age of 5 years. After its first description in 1967 by Dr Tomisaku Kawasaki, KD has become the leading cause of acquired heart diseases in children in developed nations. KD has been reported in all racial and ethnic groups but the incidence of KD is more common among Asians, and the incidence of the disease varies considerably among different Asian countries. There is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. which suggests differences of race-specific susceptibility. In Korea, from 1990s, a nationwide epidemiologic study has been conducted every 3 years by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. So the average annual incidence, 105.0/100,000 children < 5 years in recent study (2003-2005) was the second highest reported rate in the world after Japan.
Asian Continental Ancestry Group ; Child ; Developed Countries ; Epidemiologic Studies ; Epidemiology ; Ethnic Groups ; Heart ; Heart Diseases ; Humans ; Incidence ; Japan ; Korea* ; Mucocutaneous Lymph Node Syndrome* ; Prevalence

No abstract available.
Fontan Procedure* ; Heart*

No abstract available.
Fontan Procedure*

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.
Arrhythmias, Cardiac ; Arterial Pressure ; Follow-Up Studies ; Fontan Procedure* ; Freedom ; Heart ; Heart Bypass, Right ; Heterotaxy Syndrome ; Hospital Mortality ; Humans ; Incidence ; Korea* ; Longevity ; Mortality ; Multivariate Analysis ; Postoperative Period ; Preoperative Period ; Reoperation ; Risk Factors ; Survivors ; Thromboembolism

No abstract available.
Prognosis* ; Tetralogy of Fallot*

No abstract available.
Asian Continental Ancestry Group* ; Humans ; Mortality* ; Tetralogy of Fallot*

No abstract available.

Balloon dilatation of congenital stenotic lesion of the pulmonic valve has been used. Repeated balloon dilatation of restenosed lesion after previous balloon dilatation for the pulmonic stenosis is needed in some case. We treated a case of pulmonic restenosis with using three balloon catheters in a boy. The most critical problems related the valvuloplasty are severe systemic hypotension and bradycardia due to stasis of blood flow. The use of three balloon catheters instead of single or double balloons for the pulmonic stenosis could be a alternative interventional method to preserve the preexistent forward blood flow during inflation and to minimize vascular injury in children with large valve annulus.
Balloon Valvuloplasty* ; Bradycardia ; Catheters* ; Child* ; Dilatation ; Humans ; Hypotension ; Inflation, Economic ; Male ; Pulmonary Valve Stenosis* ; Vascular System Injuries