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Original Article
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...

Recent incidence of congenital heart disease in neonatal care unit of secondary medical center: a single center study
Seon Young Cho, Jin-Hee Oh, Jung Hyun Lee, Jae Young Lee, Soon Ju Lee, Ji Whan Han, Dae Kyun Koh, Chang Kyu Oh
Clin Exp Pediatr. 2012;55(7):232-237.   Published online July 17, 2012
Purpose

With feasibility in the diagnoses of congenital heart disease (CHD) in the antenatal period, we suspect changes have occurred in its incidence. No data have been reported about the current incidence of simple forms of CHD in Korea. We have attempted to assess the recent incidence and characteristics of CHD in the neonatal care unit of a secondary referral medical...

Review Article
Respiratory syncytial virus prevention in children with congenital heart disease: who and how?
Nam Kyun Kim, Jae Young Choi
Clin Exp Pediatr. 2011;54(5):197-200.   Published online May 31, 2011

Respiratory syncytial virus (RSV) is a major cause of respiratory infection in children. Most of the pediatric population have RSV infection before the age of 2, and recurrent infections are common even within one season. Chronic lung disease, prematurity, along with congenital heart disease (CHD) are major risk factors in severe lower respiratory infection. In hemo-dynamically significant CHD patients with...

Practical stepwise approach to rhythm disturbances in congenital heart diseases
June Huh
Clin Exp Pediatr. 2010;53(6):680-687.   Published online June 23, 2010

Patients with congenital heart diseases (CHD) are confronted with early- and late-onset complications, such as conduction disorders, arrhythmias, myocardial dysfunction, altered coronary flow, and ischemia, throughout their lifetime despite successful hemodynamic and/or anatomical correction. Rhythm disturbance is a well-known and increasingly frequent cause of morbidity and mortality in patients with CHD. Predisposing factors to rhythm disturbances include underlying cardiac defects,...

Clinical presentations of critical cardiac defects in the newborn: Decision making and initial management
Jae Young Lee
Clin Exp Pediatr. 2010;53(6):669-679.   Published online June 23, 2010

The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases when diagnosis and proper management are delayed. Neonates with critical CHDs may present with severe cyanosis, respiratory distress, shock, or collapse, all of which are also frequent clinical presentations of various respiratory problems or sepsis in the newborn. Early diagnosis...

Original Article
Respiratory syncytial virus infection cases in congenital heart disease patients
Woo Sup Shim, Jae Yeong Lee, Jin Yong Song, Soo Jin Kim, Sung Hye Kim, So Ick Jang, Eun Yong Choi
Clin Exp Pediatr. 2010;53(3):380-391.   Published online March 15, 2010
Purpose : Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV...
Clinical course and prognosis of hemodynamically significant congenital heart defects in very low birth weight infants
Hye Soo Yoo, Ji Eun Kim, Soo Kyoung Park, Hyun Ju Seo, Yoo Jin Jeong, Seo Heui Chio, Soo In Jeong, Sung Hoon Kim, Ji Hyuk Yang, June Huh, Yun Sil Chang, Tae Gook Jun, I Seok Kang, Won Soon Park, Pyo Won Park, Heung Jae Lee
Clin Exp Pediatr. 2009;52(4):481-487.   Published online April 15, 2009
Purpose : This study investigated the clinical course and prognostic factor of very low birth weight infants (VLBWI) with hemodynamically significant congenital heart defects (CHDs). Methods : Medical records of 1,098 VLBWI with birth weight <1,500 g who had been admitted to the neonatal intensive care unit of Samsung Medical Center from October 1994 to December 2007 were reviewed retrospectively. The...
Case Report
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
Characteristics of Pulmonary Atresia and Ventricular Septal Defect According to Morphologic Classification and Changes of Pulmonary Artery after Modified B-T Shunt
Kise Nam, Jeong Hoon Kim, Jae Young Choi, Jun Hee Sul, Sung Kue Lee, Young Hwan Park
Clin Exp Pediatr. 2004;47(3):304-309.   Published online March 15, 2004
Purpose : The purpose of this study is to help determine the optimal time and method for operation of pulmonary atresia(PA) with ventricular septal defect(VSD). Methods : Seventy patients who were diagnosed as PA with VSD in the Pediatric Department of Cardiology, Severance Hospital between May, 1991 and April, 1995, were included in our study. Subjects were divided into two groups...
Congenital Heart Disease in Jeju : Postnatal Incidence and Clinical Features
Jung Ha Lee, Gyu Hong Shim, Kyung Sue Shin, Jung Yun Hong, Yeoun Woo Kim, Seong Ho Kim, Chung Il Noh
Clin Exp Pediatr. 2004;47(3):294-303.   Published online March 15, 2004
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...
Correlation between Plasma Endothelin-1 Concentrations and Pulmonary Hypertension Associated with Congenital Heart Defects
Sang Ook Park, Su Eun Park, Hyang Jae Yoo, Young Tak Lim, Hee Ju Park
Clin Exp Pediatr. 1996;39(7):970-976.   Published online July 15, 1996
Purpose : Endothelin-1 is the most potent vasoconstrictor. This study was investigated the correlation of plasma endothelin-1 and pulmonary hypertension associated with congenital heart defect. Methods : We evaluated the concentration of ET-1 in 24 cases of congenital heart disease diagnosed at the Department of Pediatrics, Pusan National University, from December 1993 to September 1994. The patients were divided into 2 groups according to mean...
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