• Clinical and Experimental Pediatrics
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• 제55권6호
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• pp.212-214
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• 2012

Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.

• Journal of Chest Surgery
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• 제22권6호
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• pp.944-950
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• 1989

Children with congenital cardiac defects associated with high pulmonary artery pressure may die despite accurate surgery. Postoperative mortality and morbidity have been attributed to acute rises in pulmonary artery pressure and resistance. Acute pulmonary hypertensive crisis is defined as a paroxysmal event in which pulmonary arterial systolic pressure rises to or above systemic levels followed by a rapid fall in systemic pressure and a minor pulmonary hypertensive event is defined as an acute rise in pulmonary arterial pressure to more than 80 % of systemic levels but without a fall in systemic pressure. From Oct. 1988 to Jul. 1989, we experienced 23 patients who showed many pulmonary hypertensive crises after operation in the Department of Thoracic and Cardiovascular Surgery, Seoul National University Children\ulcorner Hospital. Their preoperative PAP/SAPs were 53 to 123 %[mean 93.3%] and diagnoses were VSD[7], TAPVR[5], TGA[4], AVSD[3], MS[1], DORV[1], Truncus arteriosus[1], and AP window[l]. There were 9 deaths among 23 patients and they showed many pulmonary hypertensive crisis episodes during postoperative intensive care, which was managed by sedation, hyperventilation, oxygen, and acidosis correction and which decreased after using tolazoline. In view of our experience, we recommend that pulmonary artery pressure should be monitored in congenital heart defected patient with preoperative pulmonary hypertension to confirm and to manage the pulmonary hypertensive crisis accurately and using tolazoline is helpful in the treatment of pulmonary hypertensive crisis.

• Neonatal Medicine
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• 제28권1호
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• pp.48-52
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• 2021

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by skin defects involving the epidermis, dermis, subcutaneous tissue, bone, and sometimes dura. It commonly affects the scalp in approximately 70% of cases, but the neck, trunk, and the extremities can also be affected. ACC can occur either as an isolated condition or associated with other anomalies and congenital syndromes, and it can be acquired either genetically or sporadically. Morbidity and mortality are associated with the defects of skull bone, dura, and other multiple anomalies. We herein report the case of a female infant, with a large scalp defect accompanied by a skull defect noted at birth, who developed mental retardation in the preschool years.

• Journal of Chest Surgery
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• 제21권4호
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• pp.700-705
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• 1988

A case of congenital tricuspid stenosis in 24 year old male patient is presented. The lesion was successfully corrected with prosthetic valve replacement and closure of atrial and ventricular septal defects. Especially, this is the first reported case of successful large prosthetic valve replacement to a small annulus in tricuspid valve.

• Journal of Chest Surgery
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• 제22권6호
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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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• 제10권2호
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• pp.241-249
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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.

• Asian Spine Journal
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• 제12권6호
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• pp.1028-1036
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• 2018

Study Design: Single-center, observational, case-control study. Purpose: Comparison and analysis of the metabolic and coagulative profiles in patients with idiopathic scoliosis, patients with congenital scoliosis, and healthy controls. Overview of Literature: Serum melatonin deficiency has been a controversial topic in the etiopathogenesis of scoliosis. Low bone mineral density, low vitamin D3 levels, and high parathyroid hormone levels are common metabolic abnormalities associated with scoliosis that may be responsible for its pathogenesis. In addition to metabolic defects, several studies have shown coagulation defects that either persist from the preoperative period or occur during surgery and usually lead to more than the expected amount of blood loss in patients undergoing deformity correction for scoliosis. Methods: The study population (n=73) was classified into those having congenital scoliosis (n=31), those with idiopathic scoliosis (n=30), and healthy controls (n=12). After detailed clinicoradiological evaluation of all the subjects, 10-mL blood samples were collected, measured, and analyzed for various metabolic and coagulation parameters. Results: The mean serum melatonin levels in patients with idiopathic scoliosis were significantly lower than those in the healthy controls. Although the mean serum melatonin level in the congenital group was also low, the difference was not statistically significant. Serum alkaline phosphatase and parathyroid hormone levels were higher in the scoliosis groups, whereas the vitamin D level was lower. No differences were observed in the coagulation profiles of the different groups. Conclusions: Low serum melatonin levels associated with scoliosis can be a cause or an effect of scoliosis. Moreover, low bone mineral density, high bone turn over, and negative calcium balance appear to play an important role in the progression, if not the onset, of the deformity.

• 대한간호학회지
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• 제47권2호
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• pp.178-187
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• 2017

Purpose: The purposes of this study were to identify the relationships among perceived parental bonding, illness perception, and anxiety and to determine the influences of perceived parental bonding and illness perception on anxiety in adult patients with congenital heart diseases. Methods: In this study a descriptive correlational design with survey method was utilized. The participants were 143 adult patients with congenital heart disease being cared for in the cardiology out-patient clinic of A medical center. Data were collected using the Parental Bonding Instrument, Illness Perception Questionnaire Revised Scale, and Cardiac Anxiety Questionnaire Scale. Data were analyzed using descriptive statistics, independent t-test, one-way ANOVA, Pearson correlation analysis, and hierarchial regression analyses. Results: There showed significant positive relationships of anxiety with maternal overprotection, consequences, and personal control respectively. Among predictors, maternal overprotection (${\beta}=.45$), consequence (${\beta}=.26$), and personal control (${\beta}=-.03$) had statistically significant influence on anxiety. Conclusion: Nursing interventions to decrease maternal overprotection and negative consequence, and to enhance personal control are essential to decrease the anxiety of adult patients with congenital heart diseases.

• Clinical and Experimental Pediatrics
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• 제60권8호
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• pp.237-244
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• 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 considered resistant to interventional therapies can now be managed with high success rates. The hybrid approach has enabled the overcoming of limitations inherent to percutaneous access, expanding the application of endovascular therapies as adjunct to surgical interventions to improve patient outcomes and minimize invasiveness. Percutaneous pulmonary valve implantation has become a successful alternative therapy. However, most of the current recommendations about pediatric cardiac interventions (including class I recommendations) refer to off-label use of devices, because it is difficult to study the safety and efficacy of catheterization and transcatheter therapy in pediatric cardiac patients. This difficulty arises from the challenge of identifying a control population and the relatively small number of pediatric patients with congenital heart disease. Nevertheless, the pediatric interventional cardiology community has continued to develop less invasive solutions for congenital heart defects to minimize the need for open heart surgery and optimize overall outcomes. In this review, various interventional procedures in patients with congenital heart disease are explored.

• Journal of Chest Surgery
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• 제12권2호
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• pp.113-118
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• 1979

The muscular variety of congenital ventricular septal defect [Type IV VSD] comprises from 2 to 18% of the total number of patients and has multiple defects. It is difficult to diagnose by usual angiographic study. Out of 116 cases of isolated ventricular septal defects repaired at this hospital in 20 years period from 1959 and to May 8, 1979, 2 cases had muscular defect which was treated through vertical right ventriculotomy without any problems. Single defect was found in both cases and in one case PDA was associated with Type IV VSD.