BACKGROUND: It has been reported that sudden cardiac death can occur in postoperative tetralogy of Fallot (TOF) and this episode is related to the sustained ventricular tachyarrhythmia. However, the connecting mechanism of various clinical features and sudden cardiac death is not defined yet. Based on the reports that sudden cardiac death is related to the alteration of heart rate dynamics in some diseases, investigation of heart rate dynamics in postoperative TOF seems important to reveal the mechanism of sudden cardiac death. This study was done as a part of the project to investigate the alteration of heart rate variability and to identify the factors related to the sudden cardiac death in postoperative TOF. METHODS: Among the children with postoperative TOF, 13 asymptomatic children without significant hemodynamic disturbances (Doppler velocity for tricuspid regurgitation 2.5m/sec and mild pulmonary regurgitation) and ventricular arrhythmia were selected (10 boys, 3 girls; mean age, 9 year 3 months; mean duration after operation, 7 years). We evaluated the complexity and periodic dynamics of heart rate (HR) throughout 24 hours in these children and control group (mean age, 8 year 10 months). After examining each ECG data on 24-hour Holter ECG segmented into 1-hour length, we analyzed each HR time series and quantified the overall complexity of each HR time series by its correlation dimension. In addition, the power spectrum of HR and obtained low-frequency component (0.08-0.15Hz) and high-frequency component (0.15-0.4Hz) were calculated. RESULTS: This study showed that TOF group had lower correlation dimension and a nonlinear characteristics of HR time series than control group (4.055+/-0.4134 vs. 4.9310+/-0.2054, p<0.05). However, the difference was not significant in the low- (0.9864+/-0.5598 vs. 1.5560+/-0.8325, p<0.05) and high- (1.1168+/-0.1.1448 vs. 0.9271+/-0.6528, p<0.05) frequency components. CONCLUSIONS: It can be concluded that HR time series are more regular in postoperative TOF. The result was significant in correlation dimension analysis and not in the frequency domain analysis. Although the meaning of this finding in postoperative TOF is not clear at present, this may suggest the phenomenon related to the sudden cardiac death.
Arrhythmias, Cardiac ; Child* ; Death, Sudden, Cardiac ; Electrocardiography ; Female ; Heart Rate* ; Heart* ; Hemodynamics ; Humans ; Tachycardia ; Tetralogy of Fallot* ; Tricuspid Valve Insufficiency

BACKGROUND: It has been reported that sudden cardiac death can occur in postoperative tetralogy of Fallot (TOF) and this episode is related to the sustained ventricular tachyarrhythmia. However, the connecting mechanism of various clinical features and sudden cardiac death is not defined yet. Based on the reports that sudden cardiac death is related to the alteration of heart rate dynamics in some diseases, investigation of heart rate dynamics in postoperative TOF seems important to reveal the mechanism of sudden cardiac death. This study was done as a part of the project to investigate the alteration of heart rate variability and to identify the factors related to the sudden cardiac death in postoperative TOF. METHODS: Among the children with postoperative TOF, 13 asymptomatic children without significant hemodynamic disturbances (Doppler velocity for tricuspid regurgitation 2.5m/sec and mild pulmonary regurgitation) and ventricular arrhythmia were selected (10 boys, 3 girls; mean age, 9 year 3 months; mean duration after operation, 7 years). We evaluated the complexity and periodic dynamics of heart rate (HR) throughout 24 hours in these children and control group (mean age, 8 year 10 months). After examining each ECG data on 24-hour Holter ECG segmented into 1-hour length, we analyzed each HR time series and quantified the overall complexity of each HR time series by its correlation dimension. In addition, the power spectrum of HR and obtained low-frequency component (0.08-0.15Hz) and high-frequency component (0.15-0.4Hz) were calculated. RESULTS: This study showed that TOF group had lower correlation dimension and a nonlinear characteristics of HR time series than control group (4.055+/-0.4134 vs. 4.9310+/-0.2054, p<0.05). However, the difference was not significant in the low- (0.9864+/-0.5598 vs. 1.5560+/-0.8325, p<0.05) and high- (1.1168+/-0.1.1448 vs. 0.9271+/-0.6528, p<0.05) frequency components. CONCLUSIONS: It can be concluded that HR time series are more regular in postoperative TOF. The result was significant in correlation dimension analysis and not in the frequency domain analysis. Although the meaning of this finding in postoperative TOF is not clear at present, this may suggest the phenomenon related to the sudden cardiac death.
Arrhythmias, Cardiac ; Child* ; Death, Sudden, Cardiac ; Electrocardiography ; Female ; Heart Rate* ; Heart* ; Hemodynamics ; Humans ; Tachycardia ; Tetralogy of Fallot* ; Tricuspid Valve Insufficiency

Sodium nitroprusside given by intravenous drip has been used to control hypertensive crises since 1929. Its evanescent action makes it suitable for controlled hypotension during general anesthesia with enflurane. The patients (n=10) were all undergoing Cotrel-Dubousset instrumentation for the operative treatment of scoliosis or spinal stenosis. Hemodynamic variables were obtained during administration of sodium nitroprusside. Mean arterial pressure was decreased to 50-60mmHg, while cardiac output, central venous pressure, systemic vascular resistance showed only minor changes. The average dosage of sodium nitroprusside (ug/kg/ min), plotted against age, showed a significant correlation (p<0.05, r= -0.63). The amonut of whole blood transfused during operation was 5.7+/-2.2 units (mean+/-SD) and postoperative hemoglobin and hematocrit (11.19+/-1.21g/dl, 33.49+/-3.79%, respectively, mean+/-SD) were singificantly lowered than preoperative hemoglobin and hematocrit (12.94+/-1.78g/dl, 37.22+/-4.88%, respectively, mean+/-SD) (hemoglobin: p<0.004, hematoorit: p<0.006).
Anesthesia, General* ; Arterial Pressure ; Cardiac Output ; Cardiovascular System* ; Central Venous Pressure ; Enflurane* ; Hematocrit ; Hemodynamics ; Humans ; Hypotension* ; Hypotension, Controlled ; Infusions, Intravenous ; Nitroprusside* ; Scoliosis ; Sodium* ; Spinal Stenosis ; Vascular Resistance

No abstract available.
Polymerase Chain Reaction*

OBJECTIVES: The purpose of this study is to investigate the characteristics of the temperament and character of juvenile delinquents, and to explore the effect of those factors on juvenile delinquent behavior. METHODS: 128 male and 29 female juvenile delinquents on probation, and 66 male and 69 female students in the 10th and the 11th grade, completed Junior Temperament and Character Inventory (JTCI), anxiety, depression scales and Latent Delinquency Questionnaire. RESULTS: Male juvenile delinquents showed significantly higher 'novelty seeking', and lower 'harm avoidance' and 'persistence' in JTCI subscales, compared to normal control group. Stepwise multiple-regression analysis showed that 'novelty seeking' and 'harm avoidance' were significantly predicting juvenile delinquent behavior. In the case of the female adolescents, delinquent group was significantly lower 'harm avoidance' than normal groups, and stepwise multiple regression analysis showed 'self-transcendence' was the only significant predicting variable for juvenile delinquent behavior. CONCLUSION: Based on the results, temperament was related to the juvenile delinquent behavior especially for male delinquent adolescents.
Adolescent ; Anxiety ; Depression ; Female ; Humans ; Juvenile Delinquency ; Male ; Surveys and Questionnaires ; Temperament* ; Weights and Measures

The Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive syndrome characterized by congenital deafness and cardiac phenotype (QT prolongation, ventricular arrhythmias, and sudden death). JLNS has been shown to occur due to homozygous mutation in KCNQ1 or KCNE1. There have been a few clinical case reports on JLNS in Korea; however, these were not confirmed by a genetic study. We identified compound heterozygous mutations in KCNQ1 in a 5-yr-old child with JLNS, who visited the hospital due to recurrent syncope and seizures and had congenital sensorineural deafness. His electrocardiogram revealed a markedly prolonged corrected QT interval with T wave alternans. The sequence analysis of the proband revealed the presence of novel compound heterozygous deletion/splicing error mutations (c.828-830 delCTC, p.S277del/c.921G>A, p.V307V). Each mutation in KCNQ1 was identified on the maternal and paternal side. With beta-blocker therapy the patient has remained symptom-free for three and a half years.
Asian Continental Ancestry Group/*genetics ; Child, Preschool ; Electrocardiography ; Exons ; Family ; Gene Deletion ; Heterozygote ; Humans ; Jervell-Lange Nielsen Syndrome/diagnosis/*genetics ; KCNQ1 Potassium Channel/*genetics ; Male ; Mutation ; Pedigree ; Republic of Korea

BACKGROUND AND OBJECTIVES: The relative proportion of infective endocarditis (IE) with congenital heart disease (CHD) has increased because of improved survival with CHD. This may affect the current profile of IE with CHD. The aim of this study is to assess the changing profiles of IE with CHD. SUBJECTS AND METHODS: All cases diagnosed from January 1985 to May 2006 were retrospectively reviewed and were analyzed according to the diagnosed period (period I-prior to 1995, period II-after 1996). Duke criteria were used for the definition of diagnosis. RESULTS: As a whole, 121 episodes occurred (63 episodes in period I and 58 episodes in period II). Although the mean age was similar for patients diagnosed in the two periods, the proportion of younger age patients was larger during period II. During period II, the number of non-surgical ventricular septal defect (VSD) cases decreased and the number of Rastelli procedure cases increased. Negative blood cultures were similar in patients for both periods. S. viridans was the most common causative organism in patients during both periods. Post-surgical cases and IE after a dental procedure increased during period II. The in-hospital mortality rate, total complication rate, and proportion of IE requiring early surgery were not different for patients in either period. However, the interval to early surgery was shorter for patients during period II (period I 35+/-21.1 days, period II 22+/-16.5 days, p=0.041). CONCLUSION: Our study indicates that IE in CHD is still a major problem with similar clinical features exhibited during the two different periods. The presence of non-surgical VSD as a major underlying defect indicates that one needs to pay more attention to the education of the population at risk.
Diagnosis ; Education ; Endocarditis* ; Heart Defects, Congenital* ; Heart Septal Defects, Ventricular ; Hospital Mortality ; Humans ; Population Characteristics ; Retrospective Studies*

BACKGROUND: FISH and immunohistochemistry (IHC) on formalin-fixed paraffin embedded (FFPE) tissue are currently used in the clinical laboratory to determine HER2 status in invasive breast cancer patients. Since tissue-based methods are relatively time-consuming and have a limitation for standardization of procedure, we evaluated the availability of fine needle aspirates (FNA) for the assessment of HER2 status in invasive breast cancer patients. METHODS: FNA were obtained from 51 invasive breast cancer patients and were submitted for the evaluation of HER2 status. After invasive breast cancer components were ascertained by morphological evaluation, HER2 gene amplification was evaluated by FISH. The results of HER2 FISH on FNA cells were compared with those of both FISH and IHC on corresponding FFPE tissues. FISH results were interpreted by American Society of Clinical Oncology (ASCO)/College of American Pathologists (CAP) guidelines issued in 2007. RESULTS: Of 51 FNA specimens, one was excluded due to an insufficient number of cancer cells for tests. Excluding the cases that showed 'equivocal' results, 47 (98%) out of 48 cases were concordant between the results of FISH on FNA and FISH on corresponding FFPE tissue (kappa, 0.969), and 43 (93%) out of 46 cases were concordant between the results of FISH on FNA and IHC on corresponding FFPE tissue (kappa, 0.912). CONCLUSIONS: An excellent correlation was found between FISH on FNA cells and corresponding FFPE sections. We recommend FNA specimens for more rapid determination of HER2 status by FISH, which will be helpful for patient selection for individualized therapy.
Adult ; Aged ; Aged, 80 and over ; Biopsy, Fine-Needle ; Breast Neoplasms/genetics/metabolism/*pathology ; Carcinoma, Ductal, Breast/genetics/metabolism/pathology ; Female ; Gene Amplification ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Middle Aged ; Paraffin Embedding ; Receptor, erbB-2/*genetics/metabolism ; Reproducibility of Results

Since the first descriptions of Brugada as a new clinical entity defined by sudden cardiac death in patients with typical electrocardiogram (ECG) patterns, Brugada syndrome (BS) has been increasingly diagnosed. This syndrome is known as a disease that is inherited via an autosomal dominant trait, and the SCN5A mutation has been found in 20-25% of BS patients. Because BS primarily manifests in adulthood, little information is available on BS during childhood. Although there have been several reports on adult BS in Korea, pediatric BS has not been reported. Herein, we report on childhood BS in two families. One infantile BS patient and his family had a novel SCN5A mutation (c.4035G>T, p.W1345C, heterozygote) in domain III of the sodium channel.
Adult ; Brugada Syndrome ; Death, Sudden, Cardiac ; Electrocardiography ; Humans ; Korea ; Sodium Channels ; Tachycardia, Ventricular

BACKGROUND AND OBJECTIVES: Only a few studies have specifically investigated the reasons for emergency room (ER) visits in patients with congenital heart disease (CHD). The aim of this study was to identify the major reasons for ER presentation among patients with CHD that were acutely and seriously ill at a tertiary medical center in Korea. SUBJECTS AND METHODS: All 368 admissions of patients with CHD via the ER from 2003 to 2008 were enrolled. We conducted a retrospective study with review of the medical records. RESULTS: Eighty two patients were newly diagnosed as having CHD. Their major presentations were: symptoms of heart failure (41.5%), murmur (31.7%), and cyanosis (18.3%). There were 286 visits that were cases with known CHD. Their major presentations were respiratory tract infection (24.1%, 2.7+/-4.1 years of age), dysrhythmia (16.4%, 16.7+/-9.5 years), symptoms of heart failure (14.3%, 7.6+/-9.4 years), aggravated cyanosis (5.6%, 0.8+/-1.4 years), protein-losing enteropathy (4.9%), hemoptysis (4.5%), drug side effects (4.1%), and infective endocarditis (3.0%). There were significant correlations between the age distributions and major modes of presentation. Surgical treatments were required within 1 month in 38%, and 2.7% of all patients died during hospitalization. The patient group with respiratory infections and CHD showed the highest mortality (5.8%). Atrial flutter was the most frequent arrhythmia (70.2%) and 70% of these patients were post-Fontan surgery condition. The causes of heart failure in the patients with previous surgical repair were: pulmonary hypertension, myocardial dysfunction, valve regurgitation, and uncorrected lesions. CONCLUSION: Improved understanding of the common problems in the ER can help prepare clinicians to manage patients that present with CHD.
Age Distribution ; Arrhythmias, Cardiac ; Atrial Flutter ; Cyanosis ; Emergencies ; Endocarditis ; Heart ; Heart Diseases ; Heart Failure ; Hemoptysis ; Hospitalization ; Humans ; Hypertension, Pulmonary ; Korea ; Medical Records ; Protein-Losing Enteropathies ; Respiratory Tract Infections ; Retrospective Studies