• Journal of Chest Surgery
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• v.46 no.1
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• pp.56-59
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• 2013

Cardiac surgery in neonates with congenital heart disease has progressed dramatically in the past three decades. However, low-birth-weight neonates with congenital heart disease continue to challenge the intellectual and technical skills of healthcare professionals. We present a case of a low-birth-weight neonate with pulmonary atresia and a ventricular septal defect, in whom palliation was achieved with a right ventricular outflow tract stent using a hybrid procedure.

• Journal of Korean Neurosurgical Society
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• v.37 no.1
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• pp.63-66
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• 2005

The authors report a case of Currarino triad which had a congenital anorectal stenosis associated with a sacral defect and a presacral mass. A 1-year-old female presented with constipation since birth. Neurological deficits were not found on admission. She had had a diverting colostomy due to anorectal stenosis at another hospital before admission. Lumbar X-ray films showed bony defect caudal to the third sacral vertebra. Magnetic resonance image demonstrated a round cystic pelvic mass which was connected with a dural sac via anterior sacral defect. Posterior approach with Intradural removal of the presacral cystic mass was performed and followed by anoplasty by a pediatric surgeon. The cystic mass was verified histologically as mature teratoma with cystic change. Postoperatively, the urinary function and bowel movement remained intact. Currarino triad should be suspected and evaluated physically and radiographically in a case of congenital anorectal stenosis. Prompt recognition and close cooperation between pediatric surgeons and neurosurgeons is advisable to ensure adequate surgical treatment.

• Journal of Chest Surgery
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• v.13 no.1
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• pp.34-40
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• 1980

Total 193 patients over 16 years of age who have underwent a surgical correction of congenital heart diseases during the period 1964 to September of 1979 were reviewed. 106 patients were male and 87 patients were female. 85 patients were in the ages of 16 years through 20 years. The oldest patient was 54 years old male who had atrial septal defect. The commonest defects were atrial septal defect that accounted for 66 cases [34.2%]. Ventricular septal defect was next one that accounted for 66 cases [34.2%]. Patients with tetralogy of Fallot defects were 34 cases [17.6%]. 25 cases had patent ductus arteriosus [13.0%]. Patients with pulmonary stenosis were 17 cases [8.8%] and transposition of the great arteries cases were 2 cases [1%]. There were 14 cases of operative death in this series. So operative mortality rate was 7.3%. The commonest cause of death was low output syndrome and next was renal failure. This reviewed series reveals the incidence of operable congenital heart defects appearing in adult cardiac surgical patients and an aggressive surgical approach can be justified with low operative mortality like as pediatric age group.

• Neonatal Medicine
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• v.28 no.1
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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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• v.19 no.1
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• pp.25-34
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• 1986

Recently, radionuclide angiocardiogram is one of the most common procedure for assessment of ventricular performance due to its distinctive advantages such as safety, accuracy, and ease of repeated studies. Also, measurement and comparison between pre and postoperative left ventricular ejection fraction [LVEF] are meaningful for assessing the severity of myocardial damage which occurred during open heart surgery and the status of myocardial recovery. We obtained pre and post operative LVEF using radionuclide angiocardiogram on 30 patients composed of atrial septal defect, ventricular septal defect, cyanotic congenital heart disease, and valvular heart disease who undergone the open heart surgery from March to august 1984. The study revealed that ventricular septal defect and mitral valvular heart disease showed 8.1% and 6.2% decreases of postoperative LVEF, respectively. But, there are little increases of postoperative LVEF in the atrial septal defect and cyanotic congenital heart disease. In ventricular septal defect, each group of Qp/Qs over 2.0 and systolic pulmonary artery pressure over 50mmHg showed significant 17% and 14.7% decreases of postoperative LVEF, respectively. Considering the duration of the aortic cross clamping times and closing methods of VSD, each group of duration over 30 min. and of patch closure showed 13.9% and 14.2% decreases of LVEF between pre and postoperative status respectively which was significant finding statistically.

• Archives of Craniofacial Surgery
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• v.20 no.2
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• pp.139-143
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• 2019

Here we report a case of a focal atypical proliferative nodule (PN) arising from a congenital melanocytic nevus (CMN). Diagnosis was challenging because it had both benign and malignant clinical features. Unusual histopathology, immunohistochemistry, and intraoperative findings of this atypical PN are discussed. A 5-year-old girl was admitted for a congenital $5{\times}5cm$ sized scalp mass. This hemangioma-like soft mass showed biphasic characteristics such as a slow, gradual, and benign increase in size but worrisome dural invasion with cranial bone defect. We removed the scalp mass with clear resection margins. Interoperatively, we found that the cranial bone defect had already filled. Histopathologic examination showed CMN with focal atypical PN. The nodule showed sharp demarcation and cellular pleomorphism. However, in immunohistochemical study, Ki-67 proliferation index and expression levels of protein S-100 and Melan-A were very low. These were unusual findings of atypical PNs. Despite her worrisome preoperative radiologic features, she showed an indolent clinical course compatible with previously reported biologic behavior. The patient underwent follow-up inspection with magnetic resonance imaging every 6 months for up to 3 years. The nodule appeared to be stationary at the last visit.

• Journal of Chest Surgery
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• v.17 no.1
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• pp.74-77
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• 1984

Left ventricular-Right atrial canal is a rare congenital heart disease. The vast majority of the cases reported in the literature are clinically diagnosed as atrial septal defect or ventricular septal defect. The method of choice in establishing the diagnosis of left ventricular-right atrlal canal is selective left ventriculography. Recently we experienced one case of left ventricular-right atrial canal which was diagnosed as ventricular septal defect preoperatively. The type of defect was tricuspid perforation of infravalvular type, and repaired with direct suture. Postoperative course was uneventful and discharged without complication.

• Journal of Chest Surgery
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• v.12 no.2
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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.

• Clinical and Experimental Pediatrics
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• v.55 no.8
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• pp.297-300
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• 2012

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

• Journal of Chest Surgery
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• v.53 no.1
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• pp.16-21
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• 2020

Background: Patients undergoing cardiac surgery require postoperative chest drainage. However, the drain is difficult to keep in place in children with congenital heart disease. Since 2015, at Kyungpook National University Hospital, the chest tube is removed on postoperative day 1 in patients who have undergone simple congenital cardiac surgery (i.e., closure of an atrial or ventricular septal defect). In this study, we evaluated the relationship between the duration of drain placement and the likelihood of pericardial effusion after congenital cardiac surgery. Methods: The medical records of patients who underwent closure of an atrial or ventricular septal defect at our hospital between January 2014 and December 2016 were reviewed. In total, 162 patients who received follow-up echocardiography and had information available on postoperative pericardial effusion after the repair procedure were enrolled. Results: Echocardiography was performed at a median of 5 days (range, 4 to 6 days) postoperatively before discharge from the hospital. Pericardial effusion occurred in 21 patients (13.0%), of whom only 3 (1.9%) had moderate or greater pericardial effusion, regardless of the drain duration. All patients improved during outpatient follow-up without invasive management. No patient had severe complications because of pericardial effusion. The duration of drain placement did not affect the incidence of postoperative pericardial effusion (p=0.069). Operative survival was 100%. Conclusion: Based on our study, we recommend removing the drain as soon as its role is complete, generally on postoperative day 1, because early removal does not increase the incidence of pericardial effusion in patients undergoing simple congenital cardiac surgery.