Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit.... |
Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. Their median age at the time of diagnosis was 2.5 months (range,... |
Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in We conducted a retrospective study of 41 patients with Alagille syndrome... |
Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. A retrospective analysis was conducted for a total of 13 patients with Kabuki... |
Obesity is an important risk factor for hypertension in adolescents. We investigated the relationship of obesity-related indices (body mass index [BMI], waist-to-height ratio [WHR], and body fat percentage [%BF]) with blood pressure and the hemodynamic determinants of blood pressure in Korean adolescents. In 2008, 565 adolescents, aged 12-16 years, were examined. The %BF of the participants was measured by bioelectrical impedance... |
The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Fifteen patients underwent... |
Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed... |
Our study aimed to evaluated sex differences in clinical features of obese high school students. One hundred three obese high school students (body mass index [BMI]≥85th percentile) and 51 control students (BMI<85th percentile) were enrolled in this study. Anthropometric measurements were performed. Fasting serum glucose, insulin, aspartate aminotransferase, alanine aminotransferase, total cholesterol, triglyceride, low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C),... |
Purpose : Metabolic syndrome (MS), characterized by obesity and insulin resistance, elicits risk factors such as hyperlipidemia, hypertension, and glucose intolerance with additive effects on atherosclerosis, leading to cardiovascular diseases. The purposes of this study were to evaluate the prevalence of MS among overweight and obese adolescents and to investigate the impact of obesity on the cardiovascular system. Methods : In... |
Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15- year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was... |
Purpose : Macrovascular complications are the main cause of mortality in type 1 diabetes mellitus (T1DM). The purpose of this study was to clarify the presence of early vascular changes and to assess the risk factors of macrovascular complications in young adults with T1DM diagnosed in childhood and adolescence. Methods : Seventy-two patients (23.9¡¾2.4 years) with T1DM diagnosed before 18 years... |
Purpose : Hypertension is defined as average systolic blood pressure and/or diastolic blood pressure that is ≥95th percentile for gender, age, and height on ≥three occasions. Knowing that blood pressure values increase in children as they grow older, the purposes of this study were to measure blood pressure by an oscillometric device and to determine normal values and percentile curves... |
Not a few patients in children and adolescents are suffering from right ventricular (RV) dysfunction resulting from various conditions such as chronic lung disease, left ventricular dysfunction, pulmonary hypertension, or congenital heart defect. The RV is different from the left ventricle in terms of ventricular morphology, myocardial contractile pattern and special vulnerability to the pressure overload. Right ventricular failure (RVF)... |
Purpose : This is a retrospective descriptive study to determine the incidence of congenital heart disease(CHD) in Jeju and to estimate the suitability of the Jeju population as the subject for a regional birth cohort study. Methods : All patients with CHD diagnosed by two dimensional echocardiography in Cheju National University Hospital and Halla General Hospital from January 1999 to... |
Purpose : We studied the relationship between anthracycline cumulative dose and anthracycline cardiotoxicity in childhood cancer and followed up 40 children with anthracycline cardiotoxicity. Methods : A retrospective study was performed in 154 children who received anthracycline chemotherapy between January 1995 to December 2000. Cardiotoxicity was defined when the left ventricular fractional shortening(FS) was below 26%; it was divided into two... |
Purpose : Patients with pulmonary atresia with ventricular septal defects(PAVSD) have been a formidable surgical challenge. The source of pulmonary blood flow and vascular architecture are important in managing the surgical process. This study aimed to evaluate the usefulness of echocardiography in this process. Methods : This study was prospectively designed to define the role of echocardiography in PAVSD. Non-invasive evaluations... |
Purpose : Infective endocarditis(IE) is a serious complication in children with structural heart disease. We reviewed 35 cases of IE to identify the recent changes in the pattern of preexisting heart diseases, the spectrum of causative organisms and prognosis. Methods : The clinical records of children diagnosed as IE at the Seoul National University Children`s Hospital from January 1987 through December... |
Purpose : The prevalence of congenital heart anomalies is known to be higher in patients with clefts of the lip and/or palate(CL/P). The purpose of this study was to determine the prevalence and type of congenital heart anomalies in patients with CL/P. Methods : We investigated congenital heart anomalies in 756 patients presented with CL/P from January 1986 to December... |
Purpose : Cisapride, a prokinetic agent, is widely used in preterm infants with feeding intolerance or gastroesophageal reflux. Although cisapride is regarded as a safe drug, increased QTc interval or ventricular arrhythmia has been reported in adults and neonates. So we prospectively examined the effects of cisapride on the QTc interval and QT dispersion in preterm infants. Methods : QTc interval... |
Purpose : Skin prick test and determination of allergen-specific IgE antibodies in serum are methods commonly used to diagnose allergies. Several studies indicate that skin test and specific IgE have roughly the same diagnostic precision, although discrepancies exist. The objective of this study was to evaluate the influence of total serum IgE on the relation between skin prick test and... |
Purpose : Atrial ectopic tachycardia(AET) in older children and adult is characterized by being chronic, incessant and intractable. However, the nature of infantile AET is not defined yet. The purpose of this study was to evaluate our experience of infantile AET. Methods : We retrospectively reviewed the medical records of 13 infants(mean onset age, 2.8±2.3 months) diagnosed at Seoul National University... |
Purpose : In acyanotic congenital heart disease of children with left to right shunt, 99mTc- Macroaggregate albumin(MAA) was employed to determine the scintigraphic severity of pulmonary parenchymal damage. Methods : These data of lung perfusion scan using 99mTc-MAA were compared with hemodynamic values obtained from cardiac catheterization. Results : The mean Pulmonary arterial pressure(≥30mmHg), total pulmonary resistance(≥2 Wood unit)... |
Purpose : In order to find out how frequently and what kind of extra-cardiac anomalies (CAs) is associated with congenital heart disease(CHD) in general and in each CAs, autopsies with congenital cardiovascular malformations were studied. Methods : Autopsy files of Seoul National University Children' s Hospital from 1991 to 1995 were reviewed to find cases of CHDs. Other extra-CAs were tabulated from the records... |
Purpose : Syncope is not rare and is caused by various conditions ranging from common physiologic derangements to life-threatening conditions. However, there are limited reports regarding syncope in children. We retrospectively analyzed our experiences of the syncope in children. Methods : Retrospective analysis of the medical records of patients with syncopal episodes between October 1985 and June 1996. Results : Sixty-eight patients(male; 36, female; 32,... |
Purpose : There are controversies in the operative methods of aorta with ventricular septal defect in infancy. Recent results of single stage operation for coarctation of aorta with intracardiac defects were regarded as comparable to staged operation. We evaluated our interim results after single stage operation for coarctation of aorta with large ventricular septal defect in infancy. Methods : Twenty six infants who had received... |
Two families of dilated cardiomyopathy are reported. In the first family, two sons were affected. The elder child presented with congestive symptoms at the age of 5 months and was diagnosed to have dilated cardiomyopathy. The etiology of cardiomyopathy was thought to be idiopathic until his younger brother presented with near collapse at the age of 5 days and was diagnosed to have the... |
Purpose : Hypertrophic cardiomyopathy(HCMP) is a primary cardiac muscular disease with high risk of sudden cardiac death. This study is performed to understand the clinical features of children with HCMP. Methods : Retrospective analysis of the medical records of the patients diagnosed at Seoul National University Children's Hospital between October 1985 and June 1996 was done. Excluded the patients who had... |
Methylprednisolone(MP) is administered by means of intravenous pulse therapy especially in the patients with focal segmental glomerulosclerosis(FSGS). There have been reports of its complications in a few increasing frequency. We experienced three cases of cardiaccomplications during intravenous pulsetherapy.Therewere onecaseof2¡ÆA-V block(Mobitztype I) and two cases of bradycardia and hypotension. In the former,2¡ÆA-V block in the first case deveoloped 45 hours after... |
Purpose : Branch pulmonary artery stenosis remains a clinically challenging lesion. Results of surgical angioplasty are rarely rewarding, and balloon angioplasty is not uniformly successful. So, endovascular stent has been applied recently to these lesions. But this new modality has not been full yevaluated. The aim of this study is to evaluate the short-term result of stent implantation in postoperative branch pulmonary artery stenosis. Methods :... |