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

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), also termed Bland-White-Garland syndrome, is a rare congenital cardiac anomaly characterized by myocardial ischemia which becomes symptomatic during early infancy leading to infarction, left heart failure or even death. More than 90% of patients can be dead within first year of life if untreated, so adult type ALCAPA without surgical correction is quitely rare. We present a case of a 20-year-old man with ALPACA syndrome whose diagnosis took several years to be made because he was mistaken to have organic mitral regurgitation.
Adult* ; Bland White Garland Syndrome ; Camelids, New World ; Coronary Vessels* ; Diagnosis ; Heart Failure ; Humans ; Infarction ; Mitral Valve Insufficiency ; Myocardial Ischemia ; Pulmonary Artery* ; Young Adult

PURPOSE: Kawasaki disease can cause cardiovascular complications if not properly treated from the beginning. Recently, serum amyloid A(SAA) was reported to be a predictive factor of cardiovascular diseases. Therefore, it was examined whether the existence of coronary artery abnormality in Kawasaki disease can be predicted in acute stage. METHODS: Forty nine patients who were diagnosed with Kawasaki disease between October, 2006 and May, 2007 at Yonsei University, Wonju College of Medicine were selected for this study. We reviewed results of CBC, AST, ALT, CK, LDH, total bilirubin, albumin, CRP, CK-MB, troponin-I, LDL, HDL, SAA, ESR. We divided the patients into two groups: Group A consisting of patients with coronary artery lesions, and group B consisting of patients without coronary artery lesions. RESULTS: CRP was significantly higher in group A (group A 11.0+/-7.0 mg/dL vs group B 5.3+/-5.3 mg/dL, P=0.030). SAA was slightly higher in group A but did not show any statistical significance (group A 283.8+/-357.3 microgram/mL vs group B 133.2+/-293.4 microgram/mL, P=0.128). Binary regression analysis was used to identify the significance of SAA as a predictor of coronary artery abnormality but did not find any significance (SAA OR=1.000, 95% CI=0.998-1.002, P=0.950). CONCLUSION: SAA are not significant predictors of coronary artery abnormality in Kawasaki disease but are non specific factors which increase in the acute stage.
Amyloid ; Bilirubin ; Cardiovascular Diseases ; Coronary Vessels* ; Gangwon-do ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Serum Amyloid A Protein* ; Troponin I

PURPOSE: Transcatheter closure of patent foramen ovale (PFO), atrial septal defect (ASD) and patent ductus arteriosus (PDA) is a new and less traumatic technique than open heart surgery. One of the more popular occluding devices is the Amplatzer(R) septal occluder which is made of nitinol. The present study was undertaken to evaluate the safety and release of nickel after implantation of Amplatzer(R) occluder in patients with PFO, ASD and PDA. METHODS: Random blood samples were obtained from 25 patients with Amplatzer(R) PFO, ASD, PDA occluder during 4-year and 7-month post closure period. The nickel content in the specimens was determined using atomic absorption spectrometer. RESULTS: All patients showed satisfactory clinical improvements and there was no echocardiographic evidence of complications. During the post closure, concentrations of nickel in serum were within normal range with values 0.2 ug/dL. CONCLUSION: Nickel seems to be released from Amplatzer(R) occluder. The dissolusion of nickel from Amplatzer(R) occluder is minimal and systemic rise in serum levels of nickel are within normal range. However, further studies are needed to evaluate biological effects in patients with nickel hypersensitivity.
Absorption ; Ductus Arteriosus, Patent ; Echocardiography ; Foramen Ovale, Patent ; Heart Septal Defects, Atrial ; Humans ; Hypersensitivity ; Nickel* ; Reference Values ; Septal Occluder Device ; Thoracic Surgery

PURPOSE: This study sought to analyze the safety, efficacy, and follow-up results of percutaneous closure with Amplatzer septal occulder (ASO) for secundum atrial septal defect (ASD) in less than 10 years old by comparing to those of adult. METHODS: We divided 49 enrolled subjects to two groups as childhood group (25 patients, 10< years) and adult group (24 patients, 20> years). We evaluated hemodynamic changes, including Qp/Qs, systolic right ventricular pressure, pulmonary artery pressure in pre- and post-treatment. The residual shunt rate and complications were investigated. Then, we compared the results between two groups. RESULTS: The success rate of two groups was not statistically different (100% vs 95.8%). The hemodynamic changes, including Qp/Qs, systolic right ventricular pressure, pulmonary artery pressure were statistically significant between pre- and post-treatment (all P<0.05). However, hemodynamic change difference between children and adult group was not statistically significant (all P>0.2). The residual shunt rate and complications occurrence rate were not statistically significant between two groups. CONCLUSION: The cure rate and complications by percutaneous closure of ASD with ASO were not different between in children and in adult. This procedure was an effective treatment modality for ASD in children like in adult.
Adult* ; Child* ; Follow-Up Studies ; Heart Septal Defects, Atrial* ; Hemodynamics ; Humans ; Pulmonary Artery ; Septal Occluder Device* ; Ventricular Pressure

PURPOSE: This study was aimed to assess the growth of neo-aorta and its implication to neo-aortic insufficiency in children with complete transposition of great arteries (TGA) after arterial switch operation (ASO). METHOD: We retrospectively reviewed medical records, echocardiograms and cardiac catheterization of 40 patients who had underwent ASO at Samsung Medical Center from 1995 through 2001. Pulmonary artery and aorta were evaluated regarding its stenosis and regurgitation, respectively. The growth of the neo-aortic valve, sinus of neo-aortic Valsalva, and the site of aortic anastomosis were evaluated by measuring the change of the diameter. RESULT: The mean duration of follow-up after ASO was 17.2+/-12.4 months (range 1.2-67 months). Aortic insufficiency (AI) developed in 45%, in which all were mild. The neo-aortic annulus (originally pulmonary annulus) had grown as normal pulmonic valve does do (diameter of pulmonary valve annulus preoperatively, 8.9+/-1.22 mm; postoperatively at more than 6 months, 12.8+/-2.2mm). The anastomotic site of neo-aorta showed a growth curve equivalent to that of sinotubular junction of normal aorta (preoperative diameter, 7.7+/-1.4 mm; postoperatively at more than 6months, 12.7+/-3.1mm). However, the growth rate of sinus of Valsalva showed a extremely higher compared to that of normal aorta (preoperative diameter, 10.5+/-1.2 mm; postoperatively at more than 6 months, 18.8+/-2.6 mm). No significant relations could be revealed between the change of dimension of aortic root and aortic insufficiency. CONCLUSION: Aortic insufficiency was not uncommon but mild aortic dilatation was not significantly associated with AI. Although the short term result is encouraging, long-term surveillance for aortic root dilatation and aortic insufficiency remains necessary.
Aorta ; Cardiac Catheterization ; Cardiac Catheters ; Child ; Constriction, Pathologic ; Dilatation ; Follow-Up Studies ; Humans ; Medical Records ; Pulmonary Artery ; Pulmonary Valve ; Retrospective Studies ; Sinus of Valsalva ; Transposition of Great Vessels*

PURPOSE: Resveratrol (trans-3,4',5-trihydroxystilbene), abundant in skin of grapes and red wines, has been known to protect heart cells from hypoxia/ischemia injury through its anti-oxidant properties and may also exert its cardioprotective action. There, to date, are no reports about the relationship with nitric oxide (NO)-mediated mechanism. Therefore, we investigated whether resveratrol can regulate the expression of NO synthase (NOS) in an in vitro hypoxic model of cultured H9c2 cardiomyoblasts. METHODS: The cultured H9c2 cardiomyoblasts were divided into four groups: a normal control group, a hypoxic group, two groups each treated with resveratrol before and after hypoxic insult. The control cells were placed in 5% CO2 incubator, and the hypoxic and resveratrol-treated groups were placed in 1% O2 incubator. NO activity was determined for all three isoforms of NOS; induced NOS (iNOS), endothelial NOS (eNOS), and neuronal NOS (nNOS) using real-time PCR. RESULTS: The expressions of iNOS and eNOS were decreased in the hypoxic group compared to the control group, whereas the expression of nNOS was greater in the hypoxic group than in the control group. In contrast, the group treated with resveratrol before hypoxic insult showed increased expressions of iNOS and eNOS as compared to the hypoxic group. CONCLUSION: The results of the present study demonstrate that activation of iNOS and eNOS, but not nNOS, may be one of the mechanisms involved in the protective effect on resveratrol against hypoxic myocardial injury.
Anoxia ; Heart ; Incubators ; Neurons ; Nitric Oxide Synthase* ; Nitric Oxide* ; Protein Isoforms* ; Real-Time Polymerase Chain Reaction ; Skin ; Vitis ; Wine

Echocardiography is a useful, safe and noninvasive method and is the cornerstone in diagnosis and management of children with heart disease1). Although, today more technologically advanced echocardiographic methods are used in this area, transthoracic echocardiography still has been an ideal tool for cardiac assessment, as it is noninvansive, portable, and efficacious in providing detailed anatomic, hemodynamic, and physiologic information about the pediatric heart2). And the two-dimensional echocardiography with Doppler echocardiography is the essential part of the transthoracic echocardiography. Various images should be displayed in pediatric congenital heart disease, because there is a wide spectrum of anomalies. So, standard echocardiographic images and several echocardiographic findings of heart disease in children based on two-dimensional and Doppler echocardiography in transthoracic echocardiography will be briefly presented in this paper.
Child* ; Diagnosis ; Echocardiography* ; Echocardiography, Doppler ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hemodynamics ; Humans