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Case Report
Cardiology
Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(Suppl 1):S84-S87.   Published online November 30, 2016

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....

Original Article
Cardiology
Infantile Marfan syndrome in a Korean tertiary referral center
Yeon Jeong Seo, Ko-Eun Lee, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2016;59(2):59-64.   Published online February 29, 2016
Purpose

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.

Methods

Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated.

Results

Their median age at the time of diagnosis was 2.5 months (range,...

Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center
Kyung Jin Ahn, Ja Kyoung Yoon, Gi Beom Kim, Bo Sang Kwon, Jung Min Go, Jin Su Moon, Eun Jung Bae, Chung Il Noh
Clin Exp Pediatr. 2015;58(10):392-397.   Published online October 21, 2015
Purpose

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 JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis.

Methods

We conducted a retrospective study of 41 patients with Alagille syndrome...

The strong association of left-side heart anomalies with Kabuki syndrome
Ja Kyoung Yoon, Kyung Jin Ahn, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Chung Il Noh, Jung Min Ko
Clin Exp Pediatr. 2015;58(7):256-262.   Published online July 22, 2015
Purpose

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.

Methods

A retrospective analysis was conducted for a total of 13 patients with Kabuki...

Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea
Bo Kyung Jin, Ji Seok Bang, Eun Young Choi, Gi Beom Kim, Bo Sang Kwon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Woong Han Kim
Clin Exp Pediatr. 2013;56(3):125-129.   Published online March 18, 2013
Purpose

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.

Methods

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.

Results

Fifteen patients underwent...

Case Report
Aortic valve replacement surgery for a case of infantile Takayasu arteritis
Hye Won Kwon, Yoon Jung Suh, Ji Seok Bang, Bo Sang Kwon, Gi Beom Kim, Eun Jung Bae, Woong Han Kim, Chung Il Noh
Clin Exp Pediatr. 2012;55(7):254-258.   Published online July 17, 2012

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...

A case of adolescent Kawasaki disease with Epstein-Barr virus-associated infectious mononucleosis complicated by splenic infarction
Byeong Sam Choi, Bo Sang Kwon, Gi Beom Kim, Yoon Kyung Jeon, Jung-Eun Cheon, Eun Jung Bae, Chung Il Noh, Jung Yun Choi, Yong Soo Yun
Clin Exp Pediatr. 2009;52(9):1029-1034.   Published online September 15, 2009
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...
Pheochromocytoma associated with cyanotic congenital heart disease
Seung Joon Chung, Young Ah Lee, Choong Ho Shin, Sei Won Yang, Eun Jung Bae, Jung Il Noh
Clin Exp Pediatr. 2008;51(1):93-97.   Published online January 15, 2008
Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition,...
Original Article
Follow-up Study of Children with Anthracycline Cardiotoxicity
Hyok Joo Kwon, Young Hwan Song, Soo Jung Kang, Hyoung Jin Kang, Hyoung Soo Choi, Eun Jung Bae, Hee Young Shin, Chung Il Noh, Yong Soo Yun, Hyo Seop Ahn
Clin Exp Pediatr. 2003;46(3):242-249.   Published online March 15, 2003
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...
Study for Balloon Aortic Valvuloplasty in Congenital Aortic Stenosis
In Seung Park, Do Jun Cho, Mi Young Han, Jae Young Lee, Soo Jin Kim, Mee-Hye Oh, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2001;44(2):167-176.   Published online February 15, 2001
Purpose : The aims of this study are to investigate the results of balloon aortic valvuloplasty (BAV) in congenital aortic stenosis(CAS) and, especially, to compare the results between BAV performed before two months of age(Group A) and BAV after two month of age(Group B). Methods : From January 1993 to June 2000, 14 patients who were diagnosed as CAS were treated...
Diagnostic Assessment of Pulmonary Atresia with Ventricular Septal Defect; Comparison of Echocardiogram with Cardiac Angiography
Jung Yun Choi, Jeong Jin Yu, Soo Jung Kang, Jae Seong Son, Young Mee Seo, Jin Young Song, Ho Sung Kim, Eun Jung Bae, Chung Il Noh, Yong Soo Yun
Clin Exp Pediatr. 2001;44(2):154-160.   Published online February 15, 2001
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...
Study of Congenital Mitral Stenosis Cases Requiring Surgical Correction in the First 2-Years of Life
In Seung Park, Young Seok Lee, Mi Young Han, Jae Young Lee, Soo Jin Kim, Do Jun Cho, Mee-Hye Oh, Woong-Han Kim, Young-Tak Lee, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(11):1458-1464.   Published online November 15, 2000
Purpose : Congenital mitral stenosis(CMS) is a rare anomaly accounting for 0.4-0.5% of total heart disease. CMS which cases needed surgical correction in infancy are even rare. In this study, we analyzed 11 CMS patients of less than 2 year of age who needed surgical corrections, in order to find out their diagnoses, the characteristics, the results of operation and...
Follow up of Patients with Total Anomalous Pulmonary Venous Return in Right Atrial Isomerism
Soo Jin Kim, Jae Young Lee, Mi Young Han, Do Jun Jo, In Seung Park, Mee-Hye Oh, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(11):1451-1457.   Published online November 15, 2000
Purpose : Total anomalous venous return(TAPVR) is associated in more than 60% of patients with right isomerism and can significantly complicate the management of single ventricle patients at any stage of management. We studied the results of management and sought to determine factors that may influence survival in patients with TAPVR in right atrial isomerism. Methods : Between February 1991 and...
Early Results of Stent Implantation in Branch Pulmonary Artery Stenosis after Tetralogy of Fallot Repair
Sung Kyu Lee, Sang Hun Lee, Jae Young Lee, Su Jin Kim, Mi Young Han, Do Jun Cho, In Seung Park, Eun Jung Bae, Sung Ho Kim
Clin Exp Pediatr. 2000;43(10):1343-1349.   Published online October 15, 2000
Purpose : Pulmonary artery stenosis is mainly complicated by aortopulmonary shunt or total correction of tetralogy of Fallot(TOF). Results of surgical angioplasty are poor and the success rate of balloon angioplasty is 53-72%. Endovascular stents have been applied to these lesions. The purpose of this study is to evaluate the early results of stent implantation in postoperative pulmonary artery stenosis...
Case Report
A Case of Transcatheter Occlusion of Aortopulmonary Window(APW) after APW Banding
Young Seok Lee, In Seung Park, Jae Young Lee, Soo Jin Kim, Mi Young Han, Do Jun Cho, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(9):1290-1293.   Published online September 15, 2000
Aortopulmonary window is an uncommon cardiac anomaly accounting for approximately 0.2-0.6% of all congenital cardiac anomalies in which there is a connection between the ascending aorta and pulmonary trunk. Since the first report of successful surgical repair, many investigators have advocated surgical closure of all types of aortopulmonary windows using different technique. The majority of aortopulmonary windows require surgical therapy...
Original Article
Management of Post-operative Pulmonary Artery Stenosis in Tetralogy of Fallot
Young Seok Lee, In Seung Park, Jae Young Lee, Soo Jin Kim, Mi Young Han, Do Jun Cho, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 2000;43(8):1081-1089.   Published online August 15, 2000
Purpose : Pulmonary artery stenosis is a common finding in post-operative tetralogy of Fallot (TOF), and it is one of the most frequent indication of reoperation. The objective of this study was to determine the procedural success rate of balloon angioplasty(BAP), endovascular stent, and reoperation for pulmonary artery stenosis in terms of its clinical impact on the subsequent management of...
Repair of Corrected Transposition of the Great Arteries
Soo Jin Kim, Young Seok Lee, Mi Young Han, Jae Young Lee, Do Jun Jo, In Seung Park, Eun Jung Bae, Chang-Ha Lee, Woong-Han Kim, Young-Tak Lee, Seong Ho Kim
Clin Exp Pediatr. 2000;43(8):1074-1080.   Published online August 15, 2000
Purpose : To assess the clinical characteristics and surgical approaches in different anatomical and hemodynamic types of corrected transposition of great arteries(TGA) and learn the surgical results of those patients. Methods : All 52 patients who were diagnosed as corrected TGA between December 1987 and November 1999 and their medical records were reviewed. Three groups were identified according to associated anomalies;...
Treatment of Pulmonary Atresia, Ventricular Septal Defect and Diminutive Pulmonary Arteries Comparing First Palliative Management Schemes
Jean Yoon, In Seung Park, Soo Jin Kim, Mi Young Han, Do Jun Cho, Eun Jung Bae, Young Tak Lee, Seong Ho Kim
Clin Exp Pediatr. 1999;42(6):800-806.   Published online June 15, 1999
Purpose : This report reviews an 8-year treatment of pulmonary atresia, ventricular septal defect and diminutive pulmonary arteries, comparing first palliative management schemes. Methods : Between January 1989 and March 1997, patients had their pulmonary artery anatomy evaluated before any surgical managements. Twenty-two patients had diminutive pulmonary arteries(Nakata index<90). Clinical records, hemodynamic data, and cineangiograms were examined in these patients. Results...
Balloon Valvuloplasty of Pulmonary Stenosis in Patients Younger than 6 Months of Age
Yang Park, Do Jun Cho, Woo Sub Sim, In Seung Park, Eun Jung Bae, Seong Ho Kim
Clin Exp Pediatr. 1999;42(5):631-636.   Published online May 15, 1999
Purpose : Balloon valvuloplasty(BVP) is the treatment of choice for valvular pulmonary stenosis (PS). However, this procedure was usually performed in children older than 2 years. The purpose of the present study was to assess the safety and efficacy of BVP in young infants. Methods : Retrospective analysis of the medical records of 25 infants younger than 6 months of age...
Cell Proliferation and Apoptosis in Heart of Trisomy 16 in Mice
Eun Jung Bae, Yong Soo Yun, Jung Sun Kim, Jeong-Wook Seo
Clin Exp Pediatr. 1999;42(5):621-630.   Published online May 15, 1999
Purpose : Although abnormal developments of cushion and atrioventricular septum have been suggested, the exact mechanism for the development of atrioventricular septal defect is not well known. We aimed to identify the role of cell proliferation and apoptosis on cardiac morphogenesis in trisomy 16 mice(an animal model for Down's syndrome in human). Methods : We examined the difference in cardiac...
Percutaneous Closure of Patent Ductus Arteriosus Using Coil Embolization
Mi Jung Kang, Sejung Sohn, Eun Jung Bae, In Seng Park, Seong Ho Kim
Clin Exp Pediatr. 1998;41(3):369-377.   Published online March 15, 1998
Purpose : Percutaneous closure with occluding coils has been recently described as a method of nonsurgical treatment of the small patent ductus arteriosus(PDA). The snare-assisted technique or detachable coil has been newly developed, improving coil delivery and eliminating the incidence of coil embolization. This method is also applicable to residual PDA following surgical ligation or device implantation. The study purpose...
Recent Trend of Increasing Proportion of Interventional Catheterization in Congenital Heart Disease
Sang Yup Lee, Mi Jung Kang, Eun Jung Bae, In Seung Park, Woo Sup Kim, Se Jung Sohn, Seong Ho Kim
Clin Exp Pediatr. 1997;40(4):512-518.   Published online April 15, 1997
Purpose : The proportion of interventional catheterization in congenital heart disease is being increased in Korea, especially in Sejong Heart Institute, so we performed statistical analysis on the cardiac catheterization cases. Methods : Total 233 cases of congenital heart disease confirmed by cardiac catheterization at the Sejong Heart Institute between Jaunary 1995 and June 1995 were analyzed retrospectively. Results : 1) The...
The Effects of Lipo Prostaglandin E1(EglandinⓇ) in Patients with Ductus Dependent Congenital Heart Disease
Sejung Sohn, Seong Ho Kim, Eun Jung Bae, In Seung Park
Clin Exp Pediatr. 1996;39(8):1111-1121.   Published online August 15, 1996
Purpose : The adverse reactions of prostaglandin E1(PGE1) are troublesome in the preoperative management of critical patients with ductus dependent congenital heart disease, and a preparation with less adverse reactions is preferable. The effects of Lipo PGE1, a new preparation of PGE1 contained in lipid microspheres, were compared with those of conventional PGE1(PGE1-CD). Methods : Lipo PGE1 was infused at a rate of 5...
Atrial Flutter Conversion in Infants and Children Using Transesophageal Atrial Pacing
Jae Kon Ko, Seoug Ho Kim, Eun Jung Bae, I Seok Kang, Heung Jae Lee
Clin Exp Pediatr. 1994;37(7):969-975.   Published online July 15, 1994
Atrial flutter is an infrequent, but potentially unstable tachyarrythmia that occurs in pediatric ages. Transesophageal atrial pacing was used for treatment of 10 episodes of atrial flutter in 7 patients. At the time of atrial flutter conversion, patients were 6 days to 14 years old. 6 patients had associated with congenital heart disease. The atrial cycle length of atrial flutter ranged from...
Clinical study of Kasabach-Merritt syndrome.
Eun Jung Bae, Young Ah Lee, Hee Young Shin, Hyo Seop Ahn
Clin Exp Pediatr. 1991;34(3):371-379.   Published online March 31, 1991
A retrospective analysis of clinical findings in 20 patients with Kasabach-Merritt syndrome who visited Department of Pediatrics, Seoul National University Hospital from January, 1979 to June, 1990 was performed. The average age of occurrence was 6.4 month and male to female ratio was 1: 1.2. The mean size of hemangioma was 11.0x8.5 cm and the locations were cutanous in 18 (extrem- ities 5, trunk...
Cardiac Tumor in Children.
Eun Jung Bae, Young Hwue Kim, Jung Yun Choi, Yong Seung Hwang, Hyo Seop Ahn, Yong Soo Yun
Clin Exp Pediatr. 1990;33(11):1540-1547.   Published online November 30, 1990
Primary cardiac tumor is very rare and has been a medical curiosity but is potentially curable by current management with improvements of the imaging apparatus and the surgical technique. Metastatic cardiac tumor is also very rare and is more difficult to diagnose than primary ones because of lack of specific symptoms. Twelve cases of cardiac tumor, collected from 1978 to 1989 at the Department...