• Korean Journal of Veterinary Research
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• v.56 no.3
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• pp.193-195
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• 2016

The purpose of this report is to introduce persistent left cranial vena cava (PLCVC) with persistent right aortic arch (PRAA) and patent ductus arteriosus (PDA). Case 1 was a Cocker Spaniel with PRAA and case 2 was a Maltese with PDA. PLCVC was enclosed at the sites of PRAA and PDA surgery; therefore, it was lifted dorsally during PDA and PRAA surgery. Surgery to repair congenital heart defects including PRAA and PDA is recommended for dogs that do not die of PLCVC at a young age.

• Journal of the Korea Convergence Society
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• v.10 no.3
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• pp.347-354
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• 2019

Purpose: The purpose of this study was to determine the relationships among health related quality of life and uncertainty in adult patients with congenital heart diseases. Methods: The subjects were 136 adult congenital heart disease patients at the congenital heart center out-patient clinic of the A General Hospital in S city. Data were analyzed using descriptive statistics, t-test, ANOVA, Pearson's correlation coefficient and multiple regression. Results: There were significant negative correlations of quality of life with general motor function area and uncertainty. Among predictors, gross motor(${\beta}=-.38$) and daily activity (${\beta}=-.21$) had statistically significant influences on uncertainty(F=6.15, p=.018). Conclusion: Nursing interventions to promote gross motor and daily activity might be essential for adult patients with congenital heart disease in order to reduce uncertainty.

• Journal of Yeungnam Medical Science
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• v.40 no.2
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• pp.187-192
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• 2023

Background: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. Methods: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. Results: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). Conclusion: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

• Investigative Magnetic Resonance Imaging
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• v.19 no.4
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• pp.205-211
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• 2015

The human heart is a complex organ in which many complicated congenital defects may happen and some of them require surgical intervention. Due to the vast complexity of varied anatomical presentations, establishing an accurate and consistent nomenclature system is utmost important to facilitate effective communication among pediatric cardiologists, cardiothoracic surgeons and radiologists. The Van Praagh segmental approach to the complex congenital heart disease (CHD) was developed in the 1960s and has been used widely as the language for describing complex anatomy of CHD over the decades. It utilizes a systematic and sequential method to describe the cardiac segments and connections which in turn allows accurate, comprehensive and unambiguous description of CHD. It can also be applied to multiple imaging modalities such as echocardiogram, cardiac CT and MRI. The Van Praagh notation demonstrates a group of three letters, with each letter representative for a key embryologic region of cardiac anatomy: the atria, ventricles and great vessels. By using a 3-steps approach, we can evaluate complex CHD precisely and have no difficulties in communicating with other medial colleague. This pictorial essay revisits the logical steps of segmental approach, followed by a pictorial illustration of its application.

• 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.

• Clinical and Experimental Pediatrics
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• v.58 no.7
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• pp.256-262
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• 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 syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• Journal of Chest Surgery
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• v.10 no.2
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• pp.230-240
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• 1977

Atrial septal defect is one of the most frequently encountered congenital heart disease. Up to December 31, 1976, 1682 cardiac patients received cardiac catheterization in the cardiac department of Yonsei university medical college. Out of the 1682 cardiac patients 723 cases had congenital heart disease and only 116 cases had congetial atrial septal defect. This amounted to 16.04% of all those with congenital heart disease. 58 cases of congenital atrial septal defect operated in the chest surgery department were presented. Of these 58 cases of atrial septal defect, 27 cases were male and 31 cases were female. Their ages ranged from 5 years to 54 years. The systolic pressure of the main pulmonary artery of 40 out of the 58 cases of atrial septal defect was below 40% of that of the systemic blood pressure: in 6 cases, the range of the systolic pressure of the main pulmonary artery was 50-90mmHg; in 12 cases, the range of the systolic pressure of the main pulmonary artery was 40-50mmHg. Average age of these was 30. 1 years. This study tends to show that Korean patients with atrial septal defect even though younger have a slight higher systolic pressure of the main pulmonary artery than Western patients have. The pulmonary blood is 1.5-2.5 times of systemic blood flow in 52 cases out of 58 cases of atrial septal defect.In only one of the 58 cases of atrial septal defect, the Rp was found to be as high as 45% of Rs. All other cases were below this level.51 cases had ostium secundum defect, 4 out of these cases had ostium secundum defect combined with mitral incompetence and 6 out of them had double ostium secundum defect. The remaining 7 cases had ostium primum defect. Their atrial defects were repaired under direct vision utilizing extracorporeal circulation, by hemodilution technic combined with moderate hypothermia. 44 cases [2nd atrial septal defect] were repaired by direct sutures while 14 cases, including the 7 cases ostium primum defects needed patches [1 pericardium and 13 teflon patch]. In 4 cases there were single defects while showed two defects. However the associated septal defect was so small that it could be closed by direct sutures. The size of the defect ranged between 6.0cm2and 10.0cm2 in 19 cases[33.7%]: the smallest being 0. 5cm2 and the largest 24cm2. The surgical mortality was 2 cases [3.4%]. These one case with ostium primum defect, could not be resuscitated on operation table. The cause of death in this case was myocardial failure and MI. The other, a case of ostium primum defect had a second operation on the first operative day due to massive bleeding from LV vent-line insertion site.The patient died on 26th post-operative day due to sepsis.

• Korean Journal of Veterinary Research
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• v.64 no.1
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• pp.5.1-5.6
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• 2024

A 6-month-old male Ragdoll cat presented with exercise intolerance. On physical examination, there was a grade 2/6 systolic murmur at the right apex. Diagnostic tests, including SpO₂ measurement, blood tests, radiography, echocardiography, contrast echocardiography, and electrocardiography, were performed. Severe right atrial dilation, tricuspid valve leaflets and orifice displacement, right ventricular atrialization, septal leaflet adherence, anterior leaflet tethering, and right atrioventricular junction dilation were noted on echocardiography, alongside a right-to-left atrial septal defect. Cor triatriatum dexter and left ventricular aneurysm were observed. We diagnosed this case as having Ebstein anomaly with rare congenital heart deformities; which is rare in cats.

• Journal of Chest Surgery
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• v.22 no.6
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• pp.990-995
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• 1989

Since 1984, 24 patients underwent repair of atrioventricular septal defect. Nineteen had a partial defect and 5 had a complete atrioventricular septal defect. There were 9 men and 15 women, ranging in age from 1 to 50 years [mean age, 13.3 years]. Four patients had a Downs syndrome. Additional congenital heart defects were present in 11 patients. One patient had palliative operation prior to total correction. In partial defects, the primum atrial septal defect was closed with Xenomedica patch and the mitral valve was repaired with simple closure of the septal commissure. Central incompetence from annular dilatation was repaired by a local annuloplasty. In complete defect, the septal defects were closed with two patches except one. Operative mortality was 5% in partial defects and 60% in complete defects and low cardiac output was the commonest etiology. In a mean follow-up period of 27.9 months [range, 4 to 63 months] there were no late death and no instances of late-onset complete heart block. One patient required reoperation [MVR] for residual mitral regurgitation. The majority of patients were asymptomatic and mean postop. NYHA functional class was 1.2.

• Journal of Chest Surgery
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• v.28 no.7
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• pp.698-703
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• 1995

Dextrocardia means right-sided position of the heart in the chest irrespective of the cause. For the absolute diagnosis of the dextrocardia, the segmental analysis of heart is necessary. Once the segmental analysis of the dextrocardia is made, it is often relatively easy to identify the presence of any associated defects based on conventional methods including physical examination, EKG, echocardiography, and angiocardiography. Two cases of dextrocardia with congenital heart disease were treated surgically.A eleven - months old boy was operated under diagnosis of ASD, VSD, and bilateral SVC with mirror - image dextrocardia {I,L,I} by primary closure of ASD and VSD.A twenty-four months old girl was operated under diagnosis of ASD, VSD, and PS with corrected TGA {I,D,D} by primary closure of ASD, VSD and dilatation of pulmonary stenosis. Both of them were discharged healthily after operation.