• Journal of Chest Surgery
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• v.43 no.1
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• pp.58-62
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• 2010

Rastelli repair has been considered the procedure of choice for surgically repairing transposition of the great arteries combined with ventricular septal defect and pulmonary stenosis. However, the long term results have been less than optimal and these patients who ungo this procedure can eventually display conduit obstruction, left ventricular outflow tract obstruction and arrhythmias. Many new procedures are now available and they are technically challenging for making a more normal anatomic repair. In our hospital, two patients who had a TGA with VSD and PS have been repaired with a Half Turned Truncal Switch Operation and a Lecompte maneuver in 2003 and 2006, respectively. We report on our two experiences with performing a Half Turned Truncal Switch Operation, and we discuss the changes of the biventricular outflow tract.

• Journal of Genetic Medicine
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• v.14 no.1
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• pp.43-47
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• 2017

Cat eye syndrome (CES) is a very rare chromosomal syndrome characterized by various malformations such as anal atresia, preauricular malformation, coloboma of the iris, and congenial heart and renal defects. This genetic disorder is caused by partial duplication of chromosome 22, mostly as a result of a supernumerary isodicentric marker chromosome idic(22)(q11.2). Various congenital abnormalities and extreme phenotypic variability in CES patients have been reported, which have made prenatal diagnosis of CES difficult. We report the first case diagnosed with CES prenatally by multiplex ligation-dependent probe amplification in a woman who was referred to our hospital, for a fetus presenting with heart anomaly.

• Journal of the korean academy of Pediatric Dentistry
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• v.43 no.3
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• pp.313-319
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• 2016

Wolf-Hirschhorn syndrome (WHS), associated with the deletion of the short arm of chromosome 4, causes multiple congenital malformations. Patients suffer from various deformities, including mental and growth disorders, epilepsy, hypotonia, congenital heart defects, and atypical craniofacial features. The "Greek warrior helmet appearance" is the most characteristic feature, with a prominent glabella, high arched eyebrow, broad nasal bridge, and hypertelorism. Cleft lip with or without cleft palate is observed in 30% of patients. Dental structure anomalies also exist including multiple tooth agenesis and over-retained primary molars caused by MSX1 gene impairment, and cone-shaped and taurodontic teeth. This case, a 9-year-old girl with WHS, showed intellectual disability, delayed growth development, previous occurrence of seizures, otitis media, and the typical facial features of WHS. Dental findings included multiple congenital missing teeth, over-retained primary teeth, and severe caries on the primary molars. Dental treatments were performed under general anesthesia. This report documents the characteristics of WHS, including general and oral features, and discusses the importance of oral hygiene and preventive dental management.

• Journal of Chest Surgery
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• v.31 no.5
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• pp.466-471
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• 1998

Purpose: The safety and efficacy of minimally invasive techniques in congenital heart surgery were tested in this study. Materal and method: Between July 1997 and November 1997, a total of 46 children were underwent minimally invasive cardiac operations at Seoul National University Children's Hospital. Age and body weight of the patients averaged 34.6${\pm}$41.8 (Range: 1∼148) months and 14.5${\pm}$9.9(Range: 3.0∼40.0) kg, respectively. Twenty eight patients were male. Preoperative surgical indications included 15 atrial septal defects, 25 ventricular septal defects, 1 foreign body in aorta, 3 partial atrioventricular septal defects, 1 total anomalous pulmonary venous connection(cardiac type), and 1 tetralogy of Fallot. After creating a small lower midline skin incision starting as down as possible from the sternal notch, a vertical midline sternotomy extended from xyphoid process to the level of the second intercostal space, where one of the T-, J-, I- or inverted C-shaped lower lying mini-sternotomy was completed with a creation of unilateral right or bilateral trap door sternal opening. A conventional direct aortic and bicaval cannulation was routine. Result: A mean length of skin incision was 6.1${\pm}$1.0(range: 4.0∼9.0) cm. A mean distance between the suprasternal notch and the upper most point of the skin incision was 4.0${\pm}$1.1 (range: 2.0∼7.0) cm. Mean cardiopulmonary bypass time, aortic cross-clamp time, and the operation time were 62.9${\pm}$20.0(range: 28∼147), 29.8${\pm}$12.8(range: 11∼79), and 161.1${\pm}$34.5 (range: 100-250) minutes. A mean total amount of postoperative blood transfusion was 71.0${\pm}$68.1 (range: 0∼267) cc. All patients were extubated mean 11.3${\pm}$13.8(range: 1∼73) hours after operation. A mean total amount of analgesics used was 0.8${\pm}$1.8(range: 0∼9) mg of morphine. The mean duration of stay in intensive care unit and hospital stay were 35.0${\pm}$32.2 (range: 10∼194) hours and 6.2${\pm}$2.0(range: 3∼11) days. There were no wound complications and hospital deaths. Conclusion: This short-term experience disclosed that the minimally invasive technique can be feasibly applied in a selected group of congenital heart disease as well as is cosmetically more attractive approach.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.195-202
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• 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 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.287-291
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• 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 and February 2004, 17 patients with ruptured sinus of valsalva aneurysm were retrospectively reviewed for their clinical symptoms, physical findings, past history, coexistent cardiac anormalies, surgical results, and mid-term prognosis. Results : The 17 patients included 13 men and four women, with a mean age of 30 years(10-59 years). Preoperatively accompanying cardiac anormalies were ventricular septal defect(VSD, eight cases of doubly committed juxta-arterial VSD) and aortic insufficiency(11 cases). During operations, patterns of fistulous tracts were found to be right colonary sinus-right ventricle in 13 patients, right coronary sinus-right atrium in one, noncoronary sinus - right ventricle in two, noncoronary sinus - right atrium in one, and VSD was noticed in 14 patients(all were doubly committed juxta-arterial in type). The defects were closed with a patch in 13 patients, without a patch in four, with concommitant aortic valve replacement in four and with aortic valvuloplasty in two. There were no mortalities during operations or the mid-term follow-up periods($40{\pm}49$ months). Conclusion : Because, at least in Orientals, VSD(especially doubly committed juxta-arterial) was accompanied in large numbers of patients with aneurysms of sinus valsalva, preoperative evaluations of this congenital heart disease should be made very careful. And we may need to revise the algorithm of treatment policy in small sized doubly committed juxta-arterial VSD.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.380-391
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• 2010

Purpose : Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. Methods : On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. Results : During the winter season, RSV caused 20-0% of LRI admissions in children. In patients with completely repaired simple left to right (L-R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L-R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. Conclusion : To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied.

• Journal of The Korean Dental Society of Anesthesiology
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• v.12 no.1
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• pp.45-50
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• 2012

The Fontan operation is a heart operation used to treat complex congenital heart defects like tricuspid atresia, hypoplastic left heart syndrome, pulmonary atresia and single ventricle. A single ventricle is dedicated to pumping oxygenated blood to the systemic circulation and the entire systemic venous return reaches the pulmonary arterial system without the direct influence of a pumping chamber. In the patient with Fontan operation, it is important to achieve adequate pulmonary blood flow and cardiac output in anesthetic management. In this case, a 10-year-old boy (19.6 kg, 114 cm) with cleft palate, cerebral palsy and severe mental retardation, who underwent a Fontan operation when he was 4 years old, was presented for deep sedation. Because he was suffering from eating disorder with cleft palate, the orthodontist and the plastic surgeon planned to insert intraoral orthodontic device before cleft palate repair. But it was impossible to open his mouth for alginate impression procedure. After careful pre-anesthesia evaluation we planned to administer deep sedation with propofol infusion. After Intravenous catheter insertion, we started propofol intravenous infusion with the formula of a loading dose of 1.0 mg/kg followed by an infusion rate of 6.0 mg/kg/hr with syringe pump. His blood pressure was remained around 80/40 mmHg after loss of consciousness, but he could not maintain his airway patent. So we lowered the infusion rate to 3.0 mg/kg/hr, immediately. The oxygen saturation was maintained above 95% with nasal oxygen supply, and blood pressure was maintained around 100-80/60-40 mmHg. After the sedation of 110 minutes with propofol (the infusion rate to 3.0-5.0 mg/kg/hr), he fully regained consciousness, and was discharged without complication after 1 hour observation. In case of post-Fontan patient, intravenous deep sedation with propofol was safe and effective method of behavioral management during dental treatment.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.633-636
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• 2006

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

• Journal of Chest Surgery
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• v.55 no.3
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• pp.206-213
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• 2022

Background: Surgical repair of partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) using the Warden procedure has favorable outcomes. However, there remain some concerns after the Warden procedure, such as sinoatrial nodal dysfunction and systemic or pulmonary venous stenosis. We investigated the outcomes of the Warden procedure for repair of PAPVR to the SVC. Methods: This retrospective study included 22 consecutive patients who underwent the Warden procedure for PAPVR between 2002 and 2018. The median age and body weight at operation were 27.5 months (interquartile range [IQR], 5.0-56.8 months) and 13.2 kg (IQR, 6.5-16.0 kg), respectively. The median follow-up duration was 6.2 years (IQR, 3.5-11.6 years). Results: There were no cases of early or late mortality. No patients had postoperative heart rhythm problems, except 1 patient who showed transient sinoatrial nodal dysfunction in the immediate postoperative period. Procedure-related complications requiring reintervention occurred in 5 patients, including 3 of 4 SVC stenosis cases and 2 pulmonary venous stenosis cases during follow-up. The rate of freedom from reintervention related to the Warden procedure was 75.9% at 10 years. Conclusion: In cases requiring extension or creation of an atrial septal defect to achieve a sufficient venous pathway, or interposition of an entire circumferential conduit between the SVC and right atrium due to the shortness of the SVC in the Warden procedure, stenotic complications of the venous pathway occurred. Careful observation of changes in the pressure gradient or anatomical stenosis is required in such patients.