• Clinical and Experimental Pediatrics
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• 제58권7호
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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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• 제37권1호
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• pp.27-34
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• 2004

Background: Infective endocarditis shows higher operative morbidity and mortality rates than other cardiac diseases. The vast majority of studies on infective endocarditis have been made on aortic endocarditis, with little attention having been paid to infective endocarditis on the mitral valve. This study attempts to investigate the clinical aspects and operative results of infective endocarditis on the mitral valve. Meterial and Method: The subjects of this study consist of 23 patients who underwent operations for infective endocariditis on the mitral valve from June 1995 to May 2003. Among them, 2 patients suffered from prosthetic valvular endocarditis and the other 21 from native valvular endocarditis. The subjects were evenly distributed age-wise with an average age of 44.8$\pm$15.7 (11∼66) years. Emergency operations were performed on seventeen patients (73.9%) due to large vegetation or instable hemodynamic status. In preoperative examinations, twelve patients exhibited congestive heart failure, four patients renal failure, two patients spleen and renal infarction, and two patients temporary neurological defects, while one patient had a brain abscess. Based on the NYHA functional classification, seven patients were determined to be at Grade II, 9 patients at Grade III, and 6 patients at Grade IV. Vegetations were detected in 20 patients while mitral regurgitation was dominant in 19 patients with 4 patients showing up as mitral stenosis dominant on the preoperative echocardiogram. Blood cultures for causative organisms were performed on all patients, and positive results were obtained from ten patients, with five cases of Streptococcus viridance, two cases of methicillin-sensitive Staphylococcus aureus, and one case each of Corynebacteriurn, Haemophillis, and Gernella. Operations were decided according to the AA/AHA guidelines (1988). The mean follow-up period was 27.6 $\pm$23.3 (1 ∼ 97) months. Result: Mitral valve replacements were performed on 43 patients, with mechanical valves being used on 9 patients and tissue valves on the other 4. Several kinds of mitral valve repair or mitral valvuloplasty were carried out on the remaining 10 patients. Associated procedures included six aortic valve replacements, two tricuspid annuloplasty, one modified Maze operation, and one direct closure of a ventricular septal defect. Postoperative complications included two cases of bleeding and one case each of mediastinitis, low cardiac output syndrome, and pneumonia. There were no cases of early deaths, or death within 30 days following the operation. No patient died in the hospital or experienced valve related complications. One patient, however, underwent mitral valvuloplasty 3 months after the operation. Another patient died from intra-cranial hemorrhage in the 31st month after the operation. Therefore, the valve-related death rate was 4.3%, and the valve-related complication rate 8.6% on mid-term follow-up. 1, 3-, and 5-year valve- related event free rates were 90.8%, 79.5%, and 79.5%, respectively, while 1, follow-up. 1, 3-, and 5-year valve- related event free rates were 90.8%, 79.5%, and 79.5%, respectively, while 1, 3-, and 5-year survival rates were 100%, 88.8%, and 88.8%, respectively. Conclusion: The findings suggest that a complete removal of infected tissues is essential in the operative treatment of infectious endocarditis of the mitral valve. It is also suggested that when infected tissues are completely removed, neither type of material nor method of operation has a significant effect on the operation result. The postoperative results also suggest the need for a close follow-up observation of the patients suspected of having brain damage, which is caused by preoperative blood contamination or emboli from vegetation, for a possible cerebral vascular injury such as mycotic aneurysm.

• Journal of Chest Surgery
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• 제31권12호
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• pp.1183-1194
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• 1998

Background: From Sept. 1985 to Sept. 1997, 2,000 cases of open heart surgery(OHS) were performed in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College of Medicine, Inje University. Material and Method: Among the total of 2,000 cases of OHS, 1532 cases were congenital heart disease(CHD) and 468 cases were acquired heart disease(AHD). The age distribution was 9 days(4.0kg) to 68 years in CHD and 11 to 66 years in AHD. In 1532 cases of CHD, there were 1403 acyanotic cases and 129 cyanotic cases. Result: The CHD cases consisted of 940 ventricular septal defects(61.4%), 324 atrial septal defects(21.1%), 112 tetralogy of Fallot(7.3%), 46 pulmonary stenosis(3%), 38 endocardial cushion defects(2.5%), 15 valsalva sinus ruptures(1%), 4 transposition of great arteries (0.3%), 4 double outlet right ventricles(0.3%), and etc. Corrective operations were applied for congenital heart disease with a result of 3.1% hospital mortality. Of 468 AHD, 381 cases were valvular heart diseases, 48 ischemic heart diseases, 12 cardiac tumors, 8 annuloaortic ectasias, 16 dissecting aortic aneurysms and etc. In the 381 valvular heart diseases, there were 226 single valve replacements(36 aortic valve replacements(AVR), 188 mitral valve replacements(MVR), and 2 tricuspid valve replacements(TVR), among these were 71 cases of double valve replacements(AVR & MVR), 54 cases of MVR with tricuspid valve annuloplasty(TVA), and 18 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 466. In MVR, 123 St. Jude Medical valves, 90 Carpentier-Edwards valves, 65 CarboMedics valves, 42 Sorin valves and 16 other valves were used. In AVR, 68 St. Jude Medical valves, 36 CarboMedics valves, 14 Carpentier-Edwards valves and 9 other valves were used. Coronary Artery Bypass Surgery(CABG) were performed in 48 cases. The patterns of bypass graft were 14 patients of single vessel graft, 21 patients of two vessels graft, 10 patients of three vessels graft and 3 patients of four vessels graft. Conclusion: The hospital operation mortality rate of congenital acyanotic, cyanotic and acquired heart diseases were 2.0%, 15.5%, and 5.1% respectively. The overall mortality rate was 3.6%(72/2,000).

• Journal of Chest Surgery
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• 제36권11호
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• pp.812-819
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• 2003

Background: Left ventricular dysfunction is one of the important prognostic factors of early mortality and long-term survival after valve operation. We studied the intermediate term results of mitral valve reconstruction in patients with moderate to severe left ventricular dysfunction. Material and Method: Forty four patients who underwent mitral valve reconstruction with a left ventricular ejection fraction (EF) of <45% or less (20∼45%) from April 1995 through July 2001 were reviewed retrospectively. Ages ranged from 10 to 67 years (46∼14 years) and 32 patients were in NYHA class III-IV. The mitral valve diseases were regurgitation (MR) in 28 patients, stenosis(MS) in 10, and mixed lesion in 5. The etiologies of mitral valve disease were rheumatic in 20 patients, degenerative in 14, ischemic in 5, annular dilatation in 2, congenital in 2, and endocarditis in 1. Operatively, all patients had annuloplasty and/or various valvuloplasty techniques, and a total of 52 procedures were concomitantly performed. Total cardiopulmonary bypass and aortic crossclamp time were 160$\pm$57 minutes and 112$\pm$45 minutes respectively. Result: Two operative deaths occurred as a result of left ventricular failure (4.5%). After the mean follow-up of 39 months (range, 10∼83 months), there was no late death. Transthoracic echocardiography revealed no or grade I of MR in 29 patients (72.5%) and no or mild MS in 35 patients (87.5%). The actuarial survival at 5 years was 100%. Four patients required mitral valve replacement due to progressive mitral valvular disease. The actuarial freedom from valve-related reoperation at 5 years was 84$\pm$9%. Conclusion: This study suggests that mitral valve reconstruction in patients with moderate to severe left ventricular dysfunction offers good early and intermediate survival and acceptable freedom from valve-related reoperation, and it is the strategy for effective management for these patients.

• Journal of Chest Surgery
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• 제47권4호
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• pp.344-349
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• 2014

Background: The Damus-Kaye-Stansel (DKS) procedure is a method for mitigating the risk of systemic ventricular outflow tract obstruction (SVOTO). However, there have been few reports on which surgical technique shows a better outcome. The objective of this study was to compare the outcome of the DKS procedure according to the surgical technique used. Methods: We retrospectively reviewed 12 consecutive patients who underwent the DKS procedure from March 2004 to April 2013. When the relationship of the great arteries was anterior-posterior, the double-barrel technique (group A) was performed. If the relationship was side-by-side, the ascending aortic flap technique (group B) was performed. Results: There was no early mortality and 1 late mortality in group B. There was no statistically significant difference in the median peak pressure gradient of preoperative subaortic stenosis in both groups: 14 mmHg (range, 4 to 53 mmHg) in group A and 15 mmHg (range, 0 to 30 mmHg) in group B (p=0.526). Further, a significant postoperative pressure gradient was not observed in either group A or group B. More than moderate postoperative neoaortic regurgitation was observed in 1 patient of group B; this patient underwent neoaortic valve replacement 66 months after the DKS procedure. No one had a recurrent SVOTO during follow-up. Conclusion: The DKS procedure is an effective way to minimize the risk of SVOTO, and there is little difference in the outcomes of the DKS procedure according to the surgical technique used.

• Journal of Chest Surgery
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• 제38권5호
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• pp.382-384
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• 2005

Background: It is very difficult to choose the ideal valved conduit used in right ventricle outflow reconstruction in child. We can use the cryopreserved homograft but there is a limit of application because of its difficulties in the size matching and supply capacity. The $Shelhigh^{(R)}$porcine-valved conduit is commercially available and used as an alternative choice in these days. We report two cases of early Shelhigh conduit failure in right ventricular outflow tract after Ross operation in congenital aortic stenosis.

• Journal of Chest Surgery
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• 제25권7호
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• pp.685-692
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• 1992

In spite of multiple technical advances, large amount of homologous blood transfusions usually required for open heart surgery. Because the complications associated with transfusion are increased as the number of homologous transfusion increase, especially as transfusion related acquired immunodeficiency syndrome has appeared in recent years, such risks have stimulated recent interest in the use of autologous blood. This is a report concerning 23 consecutive adult autologous donors[autologous group] who had elective cardiac surgery at the Yonsei Cardiovascular Center, Yonsei University College of Medicine, from march, 1990 to august, 1991. A similar group of 23 patients operated during the same periods without autologous blood donation was used for comparison [control group] to investigate the effect of predonated autologous blood in decreasing the need of homologous transfusion and to investigate predonation related adverse effect. Autologous group consisted of 15 men and 8 women. Control group consisted of 7 men and 16 women. There were no significant differences in mean age, hemoglobin, hematocrit, RBC count, platelet count and prothrombin time on admission between the two groups. The mean autologous blood donation in autologous group was 2.2 units. In 10[43.5%] of the 23 atuologous group patients, no homologous RBC products transfusion was required. However, all patients required homologous transfusion in control group. In autologous group, patients required less homologous RBC products than control patients[2.1 units versus 5.3 units; p<0.001]. There were no significant differences in hemoglobin, hematocrit, RBC count and platelet count between the two groups before discharge. There were no serious complications related to preoperative blood donation, although 3 patients complained of mild dizziness during donation We conclude that preoperative autologous predonation of blood is a safe and effective method for reducing homologous transfusion and is recomended in all patients undergoing elective cardiac surgery except limited contraindications such as severe aortic valve stenosis or unstable angina pectoris.

• Clinical and Experimental Pediatrics
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• 제46권7호
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Journal of Chest Surgery
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• 제36권6호
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• pp.384-390
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• 2003

Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.

• Journal of Chest Surgery
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• 제34권9호
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• pp.692-697
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• 2001

Background: The left main coronary artery surgical angioplasty has become the choice of surgical procedure in isolated LM disease. We have performed 22 cases of LMCA surgical angioplasy since 1996. We report the immediate and long term result of the surgical angioplasty with their postoperative angiography. Material and Method: Between July 1994 and October 2000, 22 patients(11 men and 11 women) were subjected to surgical patch angioplasty of the LMCA, 1 patient had an additional angioplasty performed on the proximal right coronary artery. The LMCA was approached anteriorly with or without transection of the main pulmonary artery(21 cases), and in a patient who had undergone an aortic valve replacement, LMCA was approached superiorly with transection of the ascending aorta. Additional grafting was required in 5 cases. The on-lay patch was used with autologous pericardium in 6 cases and bovine pericardium in 16 cases. Result: There was no operative mortality. There was 30~50% stenosis of the anastomosis site in 3 cases at the postop. coronary angiography. Coronary angiography was reperformed in 5 cases between 5th months and 15th months postoperatively. There was 40~60% stenosis of the anastomosis site in 2 cases. There was one death at 42nd months postoperatively but the cause was unknown. No patient complained of angina with a mean follow up of 48.$\pm$22.5 months. Conclusion: There were only several coronary angiographic results in long term follow-up but we had relatively good clinical results for LMCA surgical angioplasty in the immediate and long term. The excellence of LMCA surgical angioplasty needs the result of the long term follow-up.