• Journal of Chest Surgery
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• v.30 no.9
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• pp.869-875
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• 1997

Perimembranous ventri ular septal defects(PMVSDS) are the most common type of ventricular septal defects(VSDs) and consist morphologically of deficiency of the membranous septum and variable portions of the adjacent muscular septum. Repair of VSD has begun via a right ventriculotomy. Even with this exposure, however, it mght lead to ventricular dysfunction. Transatrial exposure of VSDs is luiown to a versatile approach to PMVSDS and even malaligunent defects can be repaired by this method. Although transatrial exposure can be improved by taking down'the atrioventricular valve at the annulus, surgeons have been hesitant to do so because of concern for valvular competence. Therefore, this study was undertaken to clarity the effects of transamlular approach of tricuspid valve (TATV) at operation of PMVSD. During last 5 years, twenty eight cases from 96 patients of PMVSD were closed by TATV and follow up study was done from 3 months to 33 months and results were obtained as follows. 1. Age at operation was fr m 4 months to 38 years and most patients(17, 62%) were above 5 years. 2. Preoperative pulmonary-systemic flow ratio(QPIQS) was ranged from 1 to 2.8 and 22 patients(79%) were less than 2. 3. Peak systolic pulmonary artery pressure was below 30mmHg in 8, 30-50mmHg in 17, above 50mmHg in 3 patients and 25 patients(89%) were less than 50mmHg. 4. Preoperative tricuspid regurgitation(TR) is none in 12, trivial in 6, mild in 3, moderate in 5, severe in 2 patients but postoperative TR was none in 18, trivial in 6, mild in 4 patients, so TR in most patients had decreased or not. 5. Indications for operation were based on the presence of a significant shunt. However, in patients with small shunts, indications for operation were included additional factors, tricuspid valve pouch, RVOT obstruction(right ventricular outflow tract obstruction), subacute bacterial endocarditis and associated anomalies. 6. There were no hospital deaths and residual shunts in postoperative echocardiography. Therefore TATV is especially a good method in PMVSn where patients have trcuspid valve pouch. And it is a safe and effective technique that improves exposure for PMVSD repair and does not adversely affect tricuspid valvular competence.

• Journal of Chest Surgery
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• v.34 no.10
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• pp.733-744
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• 2001

Background: The optimal therapeutic strategies for patients with coarctation of the aorta(CoA) and ventricular septal defect(VSD) remain controversial. This study was undertaken to determine the outcome and the need for reintervention following single-stage repair of coarctation with VSD in infants younger than 6 months. Material and Method: Thirty three consecutive patients who underwent single-stage repair of CoA with VSD, from January 1995 to December 2000, at Sejong General Hospital were reviewed retrospectively. Mean age and body weight at repair were 54$\pm$37 days(12 days-171 days) and 3.9$\pm$1.1 kg(1.5~6 kg), respectively. The surgical repair of CoA was performed under deep hypothermic circulatory arrest(CA) in the early period of the study and under regional cerebral perfusion through a direct innominate arterial cannulation without CA in the later period. The technique used in the repair of the CoA was resection and extended end-to-end anastomosis(EEEA; n=16) and extended side-to-side anastomosis(ESSA; n=2) in the early period, and resection and extended end-to-side anastomosis(EESA; n= 15) in the later period. The simultaneous closure of VSD was done with a Dacron patch(n= 16) and autologous pericardium(n=17). Aortic arch hypoplasia was present in 29 patients(88%) and its types were distal(n=18), complete(n=5), and complex(n=6)

• Journal of Sasang Constitutional Medicine
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• v.19 no.3
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• pp.293-299
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• 2007

1. Objectives The main ingredient of ephedra is ephedrine which affects on autonomic nervous system induce some adverse effects just like vasoconstriction, hypertension, tachycardia, miosis, insomnia, dizziness, headache, and etc. and heart disease. If we use Mahuang according to the Sasang constitution classification in clinic, we could not only may minimize the anxiety but maximize the potential curative value in Asthma Treatment. 2. Methods On a day three times in Mahangjungchentang taken patients who with Ventricular septal defect in the aftermath of the RBBB. We are observed that the main symptoms of change, vital sign, sleep, stool, urine, heart disease. 3. Results Teaumin with asthma, the effect of Mahuang, and the side effects are fewer. The individual effects of herbs are important. but Sasang constitutional effects are important too. 4 Conclusions Mahuang can increase heart disease. But there was a difference among Sasang constitution classification. This has no side effects from Teaumin than other constitutions. If we use Mahuang according to the Sasang constitution classification in clinic. We have an excellent effect on the treatment of asthma.

• Journal of Chest Surgery
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• v.32 no.2
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• pp.119-123
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• 1999

Background: From January 1989 to December 1996, we analyzed 22 cases of ventricular septal defect associated(VSD) with aortic valvular prolapse. Material and Method: The mean age of the patients was 7 years with a range of 6 months to 22 years . Thirteen patients were male and 9 were female. The types of VSD were Kirklin type I in 13 , Kirklin type II in 8 and Kirklin type I+II in one. Result: The preoperative echocardiographic findings were aortic valvular prolapse in 10 patients, aortic valvular prolapse associated with aortic regurgitation in 6, and only aortic regurgitation in 2. Aortic valvular prolapse were found in operation field in 4 that was not be in preoperative echcardiography. Preoperative mean Qp/Qs, systolic PAP, systolic RVP were 1.48${\pm}$0.42, 27.9${\pm}$9.87, 32.9${\pm}$10.87 mmHg, respectively. Twenty patients underwent patch closure of VSD, and two patients with moderate aortic regurgitation and prolapsed of the aortic valve underwent patch closure of VSD and aortic valvuloplasty. Short and long term echocardiographic follow-up in 8 patients who had preoperative aortic regurgitation were found to have improved or not aggravated by performing VSD patch closure only and patch closure with valvuloplasty in 2. Twelve patients who had only preoperative aortic valvular prolapse had no change in prolapsed valve in postoperative echocardiography. Conclusion: Early closure of VSD with patch is necessary in VSD with aortic valvular prolapse even in associated with mild regurgitation. But in moderate regurgitation, VSD closure with valvuloplasty is recommended.

• Clinical and Experimental Pediatrics
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• v.59 no.1
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• pp.16-23
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• 2016

Purpose: The 1p36 deletion syndrome is a microdeletion syndrome characterized by developmental delays/intellectual disability, craniofacial dysmorphism, and other congenital anomalies. To date, many cases of this syndrome have been reported worldwide. However, cases with this syndrome have not been reported in Korean populations anywhere. This study was performed to report the clinical and molecular characteristics of five Korean patients with the 1p36 deletion syndrome. Methods: The clinical characteristics of the 5 patients were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were performed for genetic diagnoses. Results: All 5 patients had typical dysmorphic features including frontal bossing, flat right parietal bone, low-set ears, straight eyebrows, down-slanting palpebral fissure, hypotelorism, flat nasal roots, midface hypoplasia, pointed chins, small lips, and variable degrees of developmental delay. Each patient had multiple and variable anomalies such as a congenital heart defect including ventricular septal defect, atrial septal defect, and patent duct arteriosus, ventriculomegaly, cryptorchism, or hearing loss. Karyotyping revealed the 1p36 deletion in only 1 patient, although it was confirmed in all 5 patients by MLPA analyses. Conclusion: All the patients had the typical features of 1p36 deletion. These hallmarks can be used to identify other patients with this condition in their early years in order to provide more appropriate care.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.660-667
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• 1998

Background: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months(range, 3 to 83). Of these patients, 28(63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis(or pulmonary atresia), 14(31.8%) had double outlet right ventricle with pulmonary stenosis(or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. Results: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions(4 cases), residual muscular ventricular septal defect(1 case), and recurrent septic vegetation(1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance(p<0.05) and all the risk factors for the recurrent pulmonary stenosis such as age, pulmonary artery index, and materials used for the pulmonary outflow tract reconstruction had no statistical significance(p>0.05). Conclusions: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.314-319
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• 1985

Nineteen patients with various types of cyanotic congenital heart disease underwent systemic-pulmonary artery shunts with a microporous polytetrafluoroethylene [PTFE] graft between September, 1983, and April, 1985. Age ranged from 3 months to 18 years, and seven of them were less than 12 months old. There were seventeen Great Ormond Street type of modified Blalock-Taussig shunts, and two central polytetrafluoroethylene shunt [ascending aorta-right pulmonary artery]. There was one postoperative death [1/19=5.3%] in a 10 Kg child born with pulmonary atresia and ventricular septal defect associated with patent ductus arteriosus. He had another anomaly of imperforated anus. Relief from cyanosis was achieved in other eighteen patients with variable degree. Eighteen survivors have been followed up from 1 month to 19 months. Clinical status, auscultation, oxygen partial pressure of arterial blood, and hemoglobin have been used to establish shunt patency in all survivors. By above criteria, all survivors have good patent shunt.

• Journal of Chest Surgery
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• v.28 no.6
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• pp.610-618
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• 1995

Three neonates with interrupted aortic arch with VSD underwent one stage repair using revised technique of cardiopulmonary bypass with short period of circulatory arrest. A left posterolateral thoracotomy was made to permit mobilization of the descending aorta and placement of polytetrafluoroethylene[PTFE graft for distal aortic perfusion. Then the patient was placed in the supine position and a median sternotomy was performed to permit the proximal dissection, VSD repair, and direct anastomosis between the ascending aorta and descending aorta. This technique has advantages to facilitate direct anastomosis between the ascending aorta and the descending aorta, to lessen circulatory arrest time, and to prevent dangerous laceration and post-operative narrowing of the thin small ascending aorta at cannulation site. There was no operative mortality but postoperative stenosis developed in one case which was relieved with balloon aortoplasty.

• Korean Journal of Veterinary Research
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• v.48 no.2
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• pp.209-213
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• 2008

A four-month-old intact femlae Abyssinian cat was present for routine health evaluation, because her littermate was recently died of ventricular septal defect. Diagnostic imaging studies showed a large caudal paracardiac mass in thoracic radiography and homogenous mass adjacent to heart in the echocardiography. Further echographic study revealed that the mass was liver and the diaphragmatic line was intact. The positive contrast celiogram revealed that no extravasation of the contrast media across the diaphragm and the prolapse of diaphragm into the pleural cavity. Based on our diagnostic imaging studies, the case was diagnosed as diaphragmatic deformity in a cat mimicking a cardiac mass.

• Journal of Chest Surgery
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• v.25 no.7
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• pp.736-738
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• 1992

We report the successful use of Nifedipine in the treatment of acute pulmonary hypertension in an young child after a cardiac operation. This patient had undergone patch closure of large ventricular septal defect. She had signs of severe pulmonary artery hypertension unresponsive to hyperventilation, oxygenation, sedation, and a myriad of vas-oactive drugs. Nifedipine, 0.3mg /kg every 4 hours, effectively treated her pulmonary artery hypertension and allowed for a smooth postoperative course and positive outcome. The drug lowered systemic diastolic pressure, but not systolic pressure.