Multiple cardiac myxomas are rare in children. However, myxomas may be lethal because of their various manifestations such as blood flow obstruction, embolization and constitutional changes. Especially, the cerebral infarction due to tumor fragmentation are more likely to be misdiagnosed of acute disseminated encephalomyelitis. We report a case of multiple cardiac myxoma complicating recurrent right hemiparesis in a 12-year-old child who at first had a wrong diagnosis of acute disseminated encephalomyelitis (ADEM). Consequently, a child who show unrepresentative symptom of ADEM, should be examined rapidly by various tools to rule out the cerebral infarction from cardiogenic cause.
Cerebral Infarction ; Child* ; Diagnosis* ; Encephalomyelitis, Acute Disseminated* ; Humans ; Myxoma* ; Paresis*

Leukocytosis and neutrophilia is common during the acute phase of Kawasaki disease whereas leukopenia is not common and severe neutropenia is rare. Severe neutropenia is defined as absolute neutrophil count less than 500/mm3. There are only few publicatons reporting of atypical Kawasaki disease with severe neutropenia. We report a case of atypical Kawasaki disease with severe neutropenia.
Leukocytosis ; Leukopenia ; Mucocutaneous Lymph Node Syndrome* ; Neutropenia* ; Neutrophils

PURPOSE: Glutathione-S-transferase (GST) is a phase II metabolism enzyme and plays an important role in the detoxification of various chemicals. Recently, the genetic background of Kawasaki disease has been investigated by some researchers and significant results were documented. To demonstrate genetic background of pathogenesis of Kawasaki disease (KD), we examined the genetic polymorphism of Glutathione-S-transferase in KD patients. METHODS: Sixty-seven Korean children with Kawasaki disease and 252 Korean healthy controls were enrolled in this study. The polymorphisms of GSTM1 and GSTT1 gene were analyzed. RESULTS: The distribution of GSTM1 polymorphism between Kawasaki disease patients and controls was not significantly different [2=0.6479, P=0.4209; odds ratios (95% confidence intervals)=0.8004 (0.4651-1.3772)]. However, the distribution of GSTT1 polymorphism was significantly different between two groups [2=18.7898, P<0.0001; odds ratios (95% confidence intervals)=4.1009 (2.0950- 8.0274)]. In the combined analysis of GSTM1 and GSTT1 genes, the frequency of both null type of GSTM1/T1 genes was significantly different from both positive type of GSTM1/T1 genes [2= 8.2528, P=0.0041; odds ratios (95% confidence intervals)=4.1486 (1.4846-11.5927)]. The group of the GSTM1 positive and GSTT null type also showed significantly different from both positive type of GSTM1/T1 genes [2=17.3479, P<0.0001; odds ratios (95% confidence intervals)=6.9143 (2.5387- 18.8314)]. CONCLUSION: These results indicates that the polymorphisms of GSTT1 gene might be a susceptible factor in development of Kawasaki disease in Koreans.
Child* ; Humans ; Metabolism ; Mucocutaneous Lymph Node Syndrome* ; Odds Ratio ; Polymorphism, Genetic

PURPOSE: Recently most of patients with Kawasaki disease (KD) get treatment within several days of illness. But, some patients still suffer from coronary complication (CC) despite early initial treatment with intravenous immune globulin (IVIG) and even the additional therapy. We investigated the risk factors of CC in non-responders to initial therapy who needed additional IVIG infusion among patients with KD. METHODS: Forty five non-responders to initial IVIG who got additional IVIG infusion were reviewed from 1996 to 2007. We devided patients into two groups with CC (group A, n=17) or without CC (group B, n=28). Clinical characteristics, timing of additional IVIG infusion and laboratory results were reviewed. And we investigated the differences between the two groups and tried to find risk factors of CC. RESULTS: In comparison between the two groups, CC, clinical characteristics and timing of IVIG infusion were not different between two groups. But, total febrile days were significantly longer and peak platelets counts were significantly higher in group A (P=0.006, P=0.013). On the logistic regression analysis, total febrile days longer than 10.5 days was the only risk factor of CC in these patients. CONCLUSION: Our results showed that patients with CC inspite of repeated IVIG therapy had longer fever duration. So, additional therapy besides re-treatment with IVIG aiming at shortening total duration of fever seems to be important in refractory KD to prevent CC.
Coronary Aneurysm ; Fever ; Humans ; Immunoglobulins, Intravenous ; Logistic Models ; Mucocutaneous Lymph Node Syndrome* ; Retreatment* ; Risk Factors

PURPOSE: In developed countries, acute rheumatic fever appears to be a vanishing disease. In Korea, the incidence and severity of acute rheumatic fever (ARF) has significantly decreased in recent 30 years. According to this report, Korea sustained low incidence of ARF. METHODS: The medical records of 5 children diagnosed as ARF from January 2000 to September 2006 were reviewed retrospectively about clinical manifestations and laboratory findings. RESULTS: The average incidence of rheumatic fever was 0.17 per annual pediatric in-ward 1,000 patients. During study period, only 1 case had a definite history of preceding infection. Among 5 patients, the incidence of major manifestations were as follows:carditis 5 cases, chorea 1 case, arthritis 1 case and erythema marginatum 2 cases. Clinical findings of carditis were cardiac murmur, cardiomegly, congestive heart failure and pericardial effusion. Significant valvular lesions were mitral and aortic insufficiency. Minor manifestations and other clinical manifestations were fever, arthralgia, dyspnea, coughing, palpitation, weakness and chest pain. Laboratory findings were increased antistreptolysin O titer, positive C reactive protein (CRP) and increased erythrocyte sedimentation rate (ESR). CONCLUSION: The incidence of ARF has reduced but rheumatic carditis varies in severity from moderate to severe cardiac involvement. For many reasons ARF is being diagnosed inappropriately resulting from lack of awareness about the disease due to rarity and secondary prophylaxis. We should be aware of acute rheumatic fever and should pay attention to the treatment of the patients with streptococcal pharyngitis.
Antistreptolysin ; Arthralgia ; Arthritis ; Blood Sedimentation ; C-Reactive Protein ; Chest Pain ; Child ; Chorea ; Cough ; Developed Countries ; Dyspnea ; Erythema ; Fever ; Heart Failure ; Heart Murmurs ; Humans ; Incidence ; Korea ; Medical Records ; Myocarditis ; Pericardial Effusion ; Pharyngitis ; Retrospective Studies ; Rheumatic Fever*

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*