• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1055-1060
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• 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 the systemic circulation (systemic RV) in congenital heart defects. Despite major progress being made, assessing the RV remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and cineangiography). Evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. While there is extensive literature on the pathophysiology and treatment of left heart failure, the data for right-sided heart failure is scarce. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. An understanding of RV physiology and hemodynamics will lead to a better understanding of current and future treatment strategies for right heart failure. This will review right-sided heart failure with the implications of volume and pressure loading of the RV in congenital heart diseases.

• Journal of Chest Surgery
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• v.14 no.1
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• pp.87-90
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• 1981

Genetic and environmental factors are the two areas which have received attention in the etiology of congenital cardiac malformation. Genetic factor in many types of congenital heart disease have not been clearly delineated. Congenital heart diseases are a heterogenous category of developmental anomalies, representing in most cases the multifactorial inheritance of threshold characters, the expression of which is the product of a genetic - environmental interaction. Recently we experienced three pairs of congenital heart disease in siblings including ventricular septal defects in twin.

• Clinical and Experimental Pediatrics
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• v.64 no.6
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• pp.269-279
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• 2021

Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) is an ancient prokaryotic defense system that precisely cuts foreign genomic DNA under the control of a small number of guide RNAs. The CRISPR-Cas9 system facilitates efficient double-stranded DNA cleavage that has been recently adopted for genome editing to create or correct inherited genetic mutations causing disease. Congenital heart disease (CHD) is generally caused by genetic mutations such as base substitutions, deletions, and insertions, which result in diverse developmental defects and remains a leading cause of birth defects. Pediatric CHD patients exhibit a spectrum of cardiac abnormalities such as septal defects, valvular defects, and abnormal chamber development. CHD onset occurs during the prenatal period and often results in early lethality during childhood. Because CRISPR-Cas9-based genome editing technology has gained considerable attention for its potential to prevent and treat diseases, we will review the CRISPR-Cas9 system as a genome editing tool and focus on its therapeutic application for CHD.

• Child Health Nursing Research
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• v.25 no.3
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• pp.262-272
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• 2019

Purpose: We aimed to identify factors influencing physical activity in adolescents with complex congenital heart disease. Methods: We recruited 92 adolescents with complex congenital heart disease from a tertiary medical center in Seoul, measured their levels of physical activity, and identified factors that influenced their physical activity levels using the Global Physical Activity Questionnaire, the New York Heart Association classification, congenital heart disease complexity, the Self-Efficacy Scale, and the Parental Bonding Instrument scale. Stepwise multiple linear regression was used to determine factors influencing physical activity. Results: Total physical activity was higher in males than in females (t=4.46, p<.001). Adolescents who participated in school physical education classes engaged in more physical activity than those who did not (t=6.77, p<.001). Higher self-efficacy (${\beta}=.41$, p<.001), male gender (${\beta}=.37$, p<.001) and participation in school physical education classes (${\beta}=.19$, p=.042) were associated with a higher likelihood of engagement in physical activity. Conclusion: It is necessary to develop nursing interventions that enhance self-efficacy in order to promote physical activity in adolescents with complex congenital heart disease. Physical activity should also be promoted in an individualized manner, taking into account gender, disease severity, and parental attitude.

• Journal of Chest Surgery
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• v.22 no.1
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• pp.95-105
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• 1989

The records of 248 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular malformation during the period of 10 years from August, 1978 to July, 1988 were reviewed. During this period, the incidence of congenital cardiovascular malformation in adult was 18.2% of 1376 total heart disease operated on and 25.5% of 986 congenital heart defects. Among them, there were 200 patients in acyanotic group and 48 patients in cyanotic group. Male versus female ratio was 1:1.28. The oldest patient was 59 years old female who had atrial septal defect. The mean age was 24.4 years old. The distribution of the lesions showed a large preponderance of atrial septal defects [37.19o] followed in frequency by ventricular septal defects [918.1%], patent ductus arteriosus [17.3%], tetralogy of Fallot [16.1%], and a variety of other complex malformations[3.2%]. In the pediatric age group, relative frequency was different from that of this adult group, showing ventricular septal defects, tetralogy of Fallot, patent ductus arteriosus and atrial septal defects in order of incidence. The hospital mortality and late mortality were 6.0% and 1.7% respectively. The causes of hospital death were low cardiac output in 10 patients, arrhythmia in 2, air embolism in 1, sepsis in 1 and respiratory failure in l. Clinical improvement upto NYHA functional class I or II postoperatively has been achieved and sustained in all patients following repair except the patients of late death and receiving reoperation. This result confirms that congenital heart defects in the adults can be corrected with a good outcome and an aggressive operative approach seems justified.

• Korean Journal of Radiology
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• v.22 no.11
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• pp.1894-1908
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• 2021

Double outlet right ventricle (DORV) is a relatively common congenital heart disease in which both great arteries are connected completely or predominantly to the morphologic RV. Unlike other congenital heart diseases, DORV demonstrates various anatomic and hemodynamic subtypes, mimicking ventricular septal defect, tetralogy of Fallot, transposition of the great arteries, and functional single ventricle. Because different surgical strategies are applied to different subtypes of DORV with ventricular septal defects, a detailed assessment of intracardiac anatomy should be performed preoperatively. Due to high spatial and contrast resolutions, cardiac CT can provide an accurate characterization of various intracardiac morphologic features of DORV. In this pictorial essay, major anatomic factors affecting surgical decision-making in DORV with ventricular septal defects were comprehensively reviewed using three-dimensional cardiac CT data. In addition, the surgical procedures available for these patients and major postoperative complications are described.

• Journal of Chest Surgery
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• v.21 no.1
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• pp.48-54
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• 1988

168 cases of open heart surgery had been performed in Korea Veterans Hospital from Aug. 1984 to Nov. 1987. There were 150 cases of congenital heart disease and 18 cases of acquired heart disease. In congenital heart cases, 123 cases [82%] were acyanotic and 27 cases [18%] were cyanotic. Common congenital defects were VSD, TOF, ASD, PS in order of frequency. There were 11 cases of operative mortality; 5 cases [4%] in acyanotic group, 6 cases [22\ulcorner6] in cyanotic group. There was no mortality in valvular heart disease group. Overall mortality rate was 6.5%.

• Journal of Chest Surgery
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• v.29 no.9
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• pp.940-944
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• 1996

Patients over 15 years of age who have undergone a surgical correction of congenital heart disease at Kyungpook University Hospital during the period of January 1990 through October 1994 have been reviewed . One hundred forty three, 22.4 % of 628 operations, which have repaired congenital heart diseases during this period were adult patients. There were 23 patients under 20 years of age, 58 between 20∼29 years, 34 between 30∼39 years, 18 between 40∼49 years, and 10 between 50∼59 years. The most common defects were atrial septal defects which accounted for 73 cases (51.1 %) and other common anomalies were ven- tricular septal defects (57 cases, 39.9 %), tetralogy of Falloffs(4 cases, 2.8%) in order of incidence. There were 10 non-fatal operative complications (6.9 %) but there was no operative mortality. This study shows the incidence of operable congenital heart diseases in adults and the fact that it could be corrected surgically with low mortality and morbidity.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.116-121
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• 1986

Patients over 15 years of age who have undergone a surgical correction of a congenital cardiac malformation during period of January 1958 through January 1986 have been reviewed. During this period there were 3957 congenital cardiac lesions consisting of 2712 acyanotic and 1245 cyanotic cases. Among them, a total of 725 adults [18.3%] with a variety of congenital heart lesions, 548 acyanotic group and 177 cyanotic group were operated on. 372 patients were male and 252 patients were female. There were 280 patients under 20 years of age, 206 between 20-24 years, 102 between 25-29 years, 48 between 30-34 and 89 over 35 years. The most common defects were atrial septal defect which accounted for 207 cases [28.6%] and other common anomalies were VSD [140 cases, 19.3%], TOF [136 cases, 18.6%], PDA [120 cases, 16.6%] and PS [33 cases, 4.6%] in order of incidence. Overall operative mortality for this series was 3.6% [1.8% of a cyanotic group and 9.0% of cyanotic group] compared with 2.8% of total cases of congenital heart disease [acyanotic group 1.1% and cyanotic group 6.5%]. This reviewed series reveals the incidence of operable congenital heart defects appearing in any adult life and demonstrates that surgical repair can be accomplished with a satisfactory low mortality rate.

• Child Health Nursing Research
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• v.28 no.3
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• pp.187-196
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• 2022

Purpose: This study aimed to clarify the factors related to mothers' decisions to provide disease explanations to their children, investigate the associations between those factors and social adaptive skills, and examine support relative to children's growth and development. Methods: Data were collected from anonymous, self-administered questionnaires answered by 71 mothers of outpatient school-aged children with congenital heart disease. The questionnaire items included characteristics of mothers and children, decision factors for providing disease explanations, and children's social adaptive skills (Asahide-Shiki social adaptive skills test). Factor analysis was performed on the decision factors, and multiple regression analysis was performed to examine the association between the identified factors and each social adaptive skill. Results: The decision factors for providing disease explanations were the mother's explanation ability, the receptive capability of the child, and the child's interest and literacy regarding the explanation. Multiple regression analysis showed that language and social life skills were significantly associated with the child's receptive capability, and language and daily life skills were significantly associated with the child's interest and literacy regarding the explanation. Conclusion: Improving children's language, social life, and daily life skills may enhance their receptive capability and literacy regarding explanations of their disease.