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Review Article
Environmental changes surrounding congenital heart disease
Eun-Young Choi
As the outcomes of patients with congenital heart disease (CHD) improve, the number of patients is accumulating and the proportion of adult CHD patients is gradually increasing. Accordingly, the proportion of typical adult diseases such as coronary arterial disease, diabetes, dyslipidemia, and hypertension among patients with CHD is increasing, as is the medical burden due to residual cardiac problems, heart...
Cardiology
Application of CRISPR-Cas9 gene editing for congenital heart disease
Heeyoung Seok, Rui Deng, Douglas B. Cowan, Da-Zhi Wang
Clin Exp Pediatr. 2021;64(6):269-279.   Published online March 2, 2021
Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) system has made a big step in the genome editing which still requires technical developments for the efficient applications in the many fields including congenital heart diseases (CHDs), closely related to the genomic abnormality. In this review, we tried to cover the most updated researches of CRISPR-Cas9 in the CHDs to understand the current technologies, eventually becoming therapeutic bases for the CHD patients.
Original Article
Neonatology (Perinatology)
New modified version of the Risk Adjustment for Congenital Heart Surgery category and mortality in premature infants with critical congenital heart disease
Young Mi Yoon, Seong Phil Bae, Yoon-Joo Kim, Jae Gun Kwak, Woong-Han Kim, Mi Kyoung Song, Seung Han Shin, Ee-Kyung Kim, Han-Suk Kim
Clin Exp Pediatr. 2020;63(10):395-401.   Published online July 15, 2020
Questions: This study aimed to describe the survival of premature infants with critical congenital heart disease (CHD) and to identify the risk factors including the new modified version of the Risk Adjustment for Congenital Heart Surgery (M-RACHS) associated with mortality.
Finding: For premature infants with critical CHD, survival rate was 76.9% and very low birth weight (VLBW), persistent pulmonary hypertension of the newborn (PPHN), bronchopulmonary dysplasia (BPD), and M-RACHS 5 or more were associated with in-hospital mortality.
Meaning: VLBW, PPHN and BPD, as well as M-RACHS≥5, were risk factors for mortality among premature infants with critical CHD.
Cardiology
Outcomes of transcatheter closure of ductus arteriosus in infants less than 6 months of age: a single-center experience
Gwang-Jun Choi, Jinyoung Song, Yi-Seul Kim, Heirim Lee, June Huh, I-Seok Kang
Clin Exp Pediatr. 2018;61(12):397-402.   Published online September 19, 2018

Purpose: Transcatheter device closure of patent ductus arteriosus (PDA) is challenging in early infancy. We evaluated PDA closure in infants less than 6 months old. Methods: We performed a retrospective review of infants less than 6 months of age who underwent attempted transcatheter device closure in our institution since 2004. To compare clinical outcomes between age groups, infants aged 6–12 months...
The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute
Moon Sun Kim, Ja Kyoung Yoon, Seong Ho Kim, Ji Seok Bang, So Ick Jang, Sang Yoon Lee, Eun Young Choi, Su Jin Park, Hye Won Kwon
Clin Exp Pediatr. 2018;61(6):187-193.   Published online June 25, 2018
Purpose

The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups.

Methods

Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the...

Review Article
Cardiology
Recent advances in pediatric interventional cardiology
Seong-Ho Kim
Clin Exp Pediatr. 2017;60(8):237-244.   Published online August 14, 2017

During the last 10 years, there have been major technological achievements in pediatric interventional cardiology. In addition, there have been several advances in cardiac imaging, especially in 3-dimensional imaging of echocardiography, computed tomography, magnetic resonance imaging, and cineangiography. Therefore, more types of congenital heart diseases can be treated in the cardiac catheter laboratory today than ever before. Furthermore, lesions previously...

Original Article
Cardiology
Impact of postoperative duration of Aspirin use on longevity of bioprosthetic pulmonary valve in patients who underwent congenital heart disease repair
Tae-Woong Hwang, Sung-Ook Kim, Sang-Yun Lee, Seong-Ho Kim, Eun-Young Choi, So-Ick Jang, Su-Jin Park, Hye-Won Kwon, Hyo-Bin Lim, Chang-Ha Lee, Eun-Seok Choi
Clin Exp Pediatr. 2016;59(11):446-450.   Published online November 18, 2016
Purpose

Generally, aspirin is used as a protective agent against thrombogenic phenomenon after pulmonary valve replacement (PVR) using a bioprosthetic valve. However, the appropriate duration of aspirin use is unclear. We analyzed the impact of postoperative duration of aspirin use on the longevity of bioprosthetic pulmonary valves in patients who underwent repair for congenital heart diseases.

Methods

We retrospectively reviewed the clinical data...

Review Article
Right ventricular failure in congenital heart disease
Young Kuk Cho, Jae Sook Ma
Clin Exp Pediatr. 2013;56(3):101-106.   Published online March 18, 2013

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression,...

Respiratory syncytial virus infection in children with congenital heart disease: global data and interim results of Korean RSV-CHD survey
Jo Won Jung
Clin Exp Pediatr. 2011;54(5):192-196.   Published online May 31, 2011

Respiratory syncytial virus (RSV) is a main cause of hospitalization for bronchiolitis and pneumonia in infants worldwide. Children with hemodynamically significant congenital heart disease (HS-CHD), as well as premature infants are at high risk for severe RSV diseases. Mortality rates for CHD patients hospitalized with RSV have been reported as about 24 times higher compared with those without RSV infection....

Congenital heart disease in the newborn requiring early intervention
Sin Weon Yun
Clin Exp Pediatr. 2011;54(5):183-191.   Published online May 31, 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary...

Original Article
Early postoperative arrhythmias after open heart surgery of pediatric congenital heart diseas
Hee Joung Choi, Yeo Hyang Kim, Joon Yong Cho, Myung Chul Hyun, Sang Bum Lee, Kyu Tae Kim
Clin Exp Pediatr. 2010;53(4):532-537.   Published online April 15, 2010
Purpose : Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. Methods : From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University...
Final height of patients with congenital heart disease
Soon Ju Lee, Young Kuk Cho, Jae Sook Ma
Clin Exp Pediatr. 2010;53(2):203-209.   Published online February 15, 2010
Purpose : Growth impairment is usually observed in children with congenital heart disease (CHD). Studies on the final height and weight of this population are insufficient. Our aim was to evaluate the final height of children with CHD and to identify the relationship between CHD and growth. Methods : We recorded the values of final height and weights of 105 CHD...
Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions
Cha Gon Lee, Su In Jeong, June Huh, I-Seok Kang, Heung Jae Lee, Ji-Hyuk Yang, Tae Gook Jun
Clin Exp Pediatr. 2010;53(2):195-202.   Published online February 15, 2010
Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21...
Acoustic variations associated with congenital heart disease
Jung Eun Oh, Yoon Mi Choi, Sun Jun Kim, Chan Uhng Joo
Clin Exp Pediatr. 2010;53(2):190-194.   Published online February 15, 2010
Purpose : To investigate the nature of deviant voice physiology in preoperative children with congenital heart disease. Methods : Ninety-four children with congenital heart disease were enrolled. Their cries and related acoustic variables (fundamental frequency, duration, noise to harmonic ratio, jitter¢¬ and shimmer) were analyzed using a multi-dimensional voice program. Results : The average fundamental frequency showed a significant decrease in patent...
The changes in signal-averaged electrocardiogram after surgical correction of congenital heart disease
Yeo Hyang Kim, Hee Jung Choi, Gun Jik Kim, Joon Yong Cho, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 2009;52(12):1364-1369.   Published online December 15, 2009
Purpose : To analyze abnormal ventricular activation in childhood congenital heart disease induced by postoperative changes in ventricular volume and pressure and ventricular scar formation using signal-averaged electrocardiography (SAECG). Methods : Fifty-two patients who had undergone open heart surgery (OHS) were enrolled. Patients were divided into the following 3 groups: right ventricular volume overload (atrial septal defect, group1), left ventricular...
Case Report
Plastic bronchitis in children: 2 cases
Yeo Hyang Kim, Hee Jung Choi, Jung Ok Kim, Myung Chul Hyun
Clin Exp Pediatr. 2009;52(7):832-836.   Published online July 15, 2009
Plastic bronchitis is a rare disorder characterized by the formation of extensive, obstructing endobronchial casts. It is associated with asthma and complex cardiac defects such as those requiring the Fontan procedure. The treatment of plastic bronchitis comprises conventional therapy involving spontaneous expectoration and bronchoscopic removal and specific therapy with several new drugs. Herein, we describe the cases of 2 patients...
Review Article
Right-sided heart failure in congenital heart diseases
Jo Won Jung
Clin Exp Pediatr. 2007;50(11):1055-1060.   Published online November 15, 2007
Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes...
Original Article
Evaluation of short-term cardiac function by tissue Doppler imaging in pre and postoperative period of congenital heart disease
Jun-Hwa Lee, Yeo-Hyang Kim, Myung-Chul Hyun, Sang-Bum Lee
Clin Exp Pediatr. 2007;50(5):476-483.   Published online May 15, 2007
Purpose : The objective of this study was to assess ventricular function by tissue Doppler imaging (TDI) in children with congenital heart disease (CHD) who have been undergoing open heart surgery (OHS) using cardiopulmonary bypass. We tried to compare the parameters of tissue Doppler imaging before and after OHS in patients with congenital heart disease. Methods : This study was conducted...
Impact of fetal diagnosis of congenital heart disease on parents
Eun Young Choi, Chang Hoon Lee, Myung Ja Yoon, Eun Sook Han, Joon Suk Hong, Yun Sook Jung, Jung Yun Choi
Clin Exp Pediatr. 2006;49(10):1073-1078.   Published online October 15, 2006
Purpose : This study was performed to assess how a fetal diagnosis of congenital heart disease affects parents, as regards pregnancy management and care of infants after birth. Methods : Database search to find out abnormal fetal echocardiography performed at Seoul National University Children's Hospital from July 1988 to June 2003 revealed 370 examinations. After excluding both arrhythmias without structural cardiac...
Relationship between depression and resilience in adolescents with congenital heart disease
Ju Ryoung Moon, Yoen Yi Jung, June Huh, I-Seok Kang, Seung Woo Park, Ji-Hyuk Yang, Tae-Gook Jun, Myung Ja Kim, Heung Jae Lee
Clin Exp Pediatr. 2006;49(5):523-528.   Published online May 15, 2006
Purpose : The purpose of this study was to investigate the relationship between depression and resilience in adolescents with congenital heart disease(CHD) and to identify the variables associated with depression. Methods : The Resilience Scale(cronbach’s α=0.92), Children's Depression Inventory(cronbach’s α= 0.72) and Maternal Behavior Research Instrument(cronbach’s α=0.88) were applied and analyzed to assess depression and resilience among 231 adolescents after...
Clinical features and surgical results of ruptured sinus of valsalva aneurysm
Tae Ho Lee, Dong Won Lee, Joon Yong Cho, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 2006;49(3):287-291.   Published online March 15, 2006
Purpose : Aneurysms of sinus valsalva are rare anormalies thought to be primarily congenital in origin, progressing into death by acute heart failure in cases of rupture. Surgical correction is the only method of treatment. With these clinical implications, we reviewed the clinical characteristics and surgical results of patients with ruptured sinus of valsalva aneurysm. Methods : Between January 1991...
Usefulness of Intraoperative Transesophageal Echocardiography
Dong Won Lee, Byung Mok Kim, Sei Young Kim, Yeo Hyang Kim, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 2004;47(12):1281-1286.   Published online December 15, 2004
Purpose : This study was undertaken to learn the usefulness, cost-effectiveness and safety of intraoperative transesophageal echocardiography(ITEE) during open heart surgery for congenital heart diseases. Methods : The usefulness and safety of ITEE were assessed in 254 patients who underwent open heart surgery for congenital heart diseases from January 1, 2001 to June 30, 2003, with retrospective chart reviews and a...
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...
Case Report
A Case of Isolated Right Ventricular Hypoplasia without Tricuspid Atresia or Pulmonary Atresia
Eui Jun Yang, Sun Young Kim, Sank Ook Nam, Hee Ju Park, Nam Hee Kwak
Clin Exp Pediatr. 1999;42(7):1026-1030.   Published online July 15, 1999
Isolated right ventricular hypoplasia, unassociated with severe pulmonary or tricuspid valvar malformations, is a rare primary congenital cardiac anomaly in which of the trabecular portion of right ventricle fails to develop. An atrial septal defect or a patent foramen ovale serves as an escape valve. We observed a 2-day-old neonate with this disorder who suffered from cyanosis. The diagnosis was...
Original Article
Assessment of Regional Wall Motion of the Left Ventricle in Children with Congenital Heart Disease
Chang Hee Han, Jun Ho Huh, Un Seok Nho, Byung Ho Choi, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 1999;42(6):807-816.   Published online June 15, 1999
Purpose : To assess wall motion of the left ventricle in the various forms of congenital heart disease and to select appropriate methods by which to gauge left ventricular wall motion. Methods : We evaluated left ventricular function, including volume, global ejection fraction, and regional wall motion(Centerline method, Local Ejection Fraction, Curved Perimeter method and Radial method). The evaluation was done...
The Assessment of Pulmonary Parenchymal Damage in Congenital Heart Disease using Lung Perfusion Scan
Hong Ryang Gil, Jae Goo Lee, Kang Uk Lee, Eun Ho Yi, Myung Ja Yun, Chung Il Noh, Jung Yeon Choi, Yong Soo Yun
Clin Exp Pediatr. 1998;41(5):640-645.   Published online May 15, 1998
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)...
Incidence of Congenital Heart Disease in Neonate by Color Doppler Echocardiography
Tae Chan Kwon, Joon Sik Kim, Sang Lak Lee, Myung Sung Kim, Geun Soo Park, Chun Soo Kim, In Joo Kim, Bin Ahn
Clin Exp Pediatr. 1998;41(3):363-368.   Published online March 15, 1998
Purpose : Many reports exist about the frequency of congenital heart disease(CHD) in the world, but according to the definition of CHD and subjects of study group, many differences existed between the reports. We conducted this study to evaluate the incidence and frequency of CHD by color Doppler echocardiography in neonate, and to suggest a preliminary model of incidence of...
Congenital Heart Disease and Associated Extracardiac Anomalies in Autopsies
Soon Seong Park, Myeong Ja Yoon, Jeong Sun Kim, Jeong Wook Seo, Chung Il Noh, Jung Yun Choi, Yong Soo Yoon, Byung Il Kim, Joong Hwan Choi, Chong Ku Yoon
Clin Exp Pediatr. 1998;41(3):354-362.   Published online March 15, 1998
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...
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...
Diagnosis and Treatment of Fetal Cardiac Anomalies
Nam Su Kim, Myung Kul Yum, Hahng Lee, Sung Ro Jung, Jung Bae Yoo, Jae Ek Lee
Clin Exp Pediatr. 1996;39(10):1395-1404.   Published online October 15, 1996
Purpose : Fetal echocardiography is used for the prenatal diagnosis of congenital heart disease. Fetal echocardiography allows decisions to be made in advance where the fetal abnormality is not compatible with life after delivery or where early correction is required. Our fetal echocardiographic experience was analysed retrospectively to guide future clinical application. Methods : 138 mothers had fetal echocardiography from April 1992 to December...
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