• Journal of Chest Surgery
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• v.36 no.2
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• pp.73-78
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• 2003

The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. Material and Method: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8 :-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1 Result: There were 3 early and late deaths respectively Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1) In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia. Conclusion: Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However, more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class, Although the results were not satisfactory enough in all patients.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.597-603
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• 2009

Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

• Journal of Chest Surgery
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• v.35 no.10
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• pp.750-754
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• 2002

Primary cardiac tumors are rare and about 20~25% of primary cardiac tumors are malignant. Moreover, primary malignant mesenchymoma of the heart is extremely rare. Recently, we have experienced two cases of cardiac malignant mesenchymoma. In the first case, malignant mesenchymoma which was originated from the posterior wall of the left atrium obstructing the mitral orifice was revealed pathologically in a 61-year-old woman with mitral regurgitation. The mass, which was 2.7$\times$3.7cm in size on the posterior wall of left atrium, was extended to the posteromedial commissure and annulus of the mitral valve. The mass was resected partially without excision of the left atrial free wall. She was discharged after 30 days without any problems and she received chemotherapy and followed up for 19months. The second case was a 4$\times$5cm in size, friable, yellow-whitish multilobulated mass in the left atrium which was originated from the left lower pulmonary vein. Multiple minor tumor nodules were found in the wall of the left atrium and the posterior leaflet of mitral valve. Partial mass excision and mitral valve replacement were performed.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.136-141
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• 2003

We assessed the intermediate-term result of tricuspid annuloplasty (TAP) for tricuspid valve regurgitation (TR) associated with congenital heart disease in adults. Risk factors for residual TR were also analysed. Material and Method: From August 1989 to June 2001, seventy three adult patients, 51 females and 22 males, underwent TAP for TR associated with various congenital heart disease. Their age ranged from 46 years to 73 years (mean:43). Associated heart anomalies were atrial septal defect (55), ventricular septal defect (6), partial anomalous pulmonary venous return (4) and others (8). Preoperative and post-operative TR velocities were 3.25 m/sec and 2.56 m/sec respectively, and the types of TAP were De Vega in 43, Kay in 18 and Ring annuloplasty in 12. Postoperative follow-up duration was 2,347 patient-month (mean: 32.6 months), and 134 two-dimensional echocardiographic examinations were done during this period. Residual TR greater than III/IV was considered as TAP failure. Result: TAP failure was observed in 7 patients (9.6%), and one patient among them underwent tricuspid valve replacement. Risk factors for TAP failure were diagnosis other than atrial septal defect (p=0.001), preoperative (p=0.038) and postoperative (p=0.028) high TR velocity. There was no statistical significance in terms of TAP methods. Conclusion: Careful evaluation of valve morphology and aggressive surgical intervention are mandatory for the repair of TR with preoperative or residual RV pressure overload.

• Journal of Yeungnam Medical Science
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• v.10 no.1
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• pp.237-244
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• 1993

A free-floating ball thrombus in the left atrium is a rare complication of the mitral valvular disease. A 53-year-old man was admitted for pain and paresthesia on both legs. On admission he had auscultatory sign of mitral stenosis and mitral regurgitation, and the roentgenogram of his chest revealed a slight pulmonary venous congestion, enlargement of the pulmonary conus and cardiomegaly. Laboratory findings including complete blood counts, coagulation studies and blood chemistry were normal. An echocardiographic examination revealed a mitral stenosis and a free-floating ball thrombus in the left atrium. We performed the emergent open heart surgery for removal of the ball thrombus and mitral replacement successfully with Duromedics 29 mm valve. The size of thrombus was $39{\times}32{\times}30$ mm.

• Journal of Korean Academy of Fundamentals of Nursing
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• v.7 no.2
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• pp.256-270
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• 2000

Invasive hemodynamic monitoring has become a valuable assessment parameters in critical care nursing in patients undergoing open heart surgery patients. During cardiac surgery, the Swan Ganz catheter is placed in the pulmonary artery. Critical care nurses routinely obtain cardiac output, cardiac index, and pulmonary arterial pressure in these patients. Traditionally, patients are positioned flat and supine for cardiac output measurement. Numerous studies have dealt with the effects of changing position on the hemodynamic variables. However, there are a few studies dealing with patients who undergo cardiac surgery in Korea. Thus, the purpose of this study was to determine the effects of changing position on cardiac output, PAP, CVP, BP, HR and discomfort in patients after cardiac surgery. A sample of 21 adults who had CABG and/or valve replacement with Swan Ganz catheters in place was studied. The data were collected in the cardiac ICU of a university hospital in Seoul during the period from July 28, 1999 to August 30. 1999. In this study, the independent variable is patient position in the supine, 30 degree, and 45 degree angles. Dependent variables are C.O., C.I., CVP, PAP, MAP, HR and patients' perceived discomforts. Subject discomfort was measured subjectively by visual analogue scale. Other hemodynamic data where collected by the thermodilution method and by direct measurement. The data were analyzed by percentile, t-test, ANOVA, Linear regression analysis using SPSS-/WIN program. The results are as follows : 1) Changes in cardiac output were absent in different angle positions, 0, 30. 45 degrees(F=.070, P=.932). Changes in cardiac index were absent in different angle positions, 0. 30, 45 degrees(P>.05). 2) Changes in central venous pressure were absent in different angle positions, 0, 30, 45 degree(P>.05). 3) PAP had no change in different angle 0, 30, 45 degree positions; systolic PAP(P>.05), diastolic PAP(P>.05). 4) Changes in systolic blood pressure were absent in different angle positions, 0, 30, 45 degree(P>.05). 5) Changes in heart rates were absent in different angle positions, 0, 30, 45 degree(P>.05). 6) Patients' perceived discomfort was absent in different angle positions, 0, 30, 45 degree(p<.05). In conclusion, critical care nurses can measure C.O., C.I., PAP, BP, & CVP in cardiac surgery patients at 30 degree or 45 degree positions. This can improve the patients' comfort.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.24-33
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• 2016

Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

• Journal of Chest Surgery
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• v.34 no.9
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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.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.271-276
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• 2005

We analysed differences in operative methods and postoperative outcome according to the severity of preoperative cyanosis in adult ToF (Tetralogy of Fallot) patients. Material and Method: From August 1989 to June 2001, thirty three adult patients, 18 females and 15 males, underwent total correction for ToF. Their age ranged from 15 years to 54 years (median: 34). Patients were divided into 2 groups by preoperative $SaO_2$ (arterial oxygen saturation): group I$(n=cyanotic,\;SaO_2\;\geq94\%)$ and group II $(acyanotic,\; SaO_2\geq95%)$. Preoperative median hemoglobin level was higher in group I compared to group II (17.5 g/dl vs 15 g/dl). Postoperative follow-up duration ranged from 1 to 94 months (670 patient-month, median: 14 months), and 63 two-dimensional echocardiographic examinations were done during this period. Result: There were no early or late mortality. With regard to RVOT (right ventricular outflow tract) reconstruction, trans-annular patch and RV-PA extracardiac conduit were used in 7 and 3 patients respectively, and all of them belonged to group I. In group I, cardiopulmonary bypass time, aortic cross-clamping time, ICU day, hospital day were significantly longer than in group II, and postoperative inotropic support was significantly greater than in group II. There was no ventricular arrhythmia in both groups, and one patient in group I suffered from atrial arrhythmia, which was resolved spontaneously after tricuspid and pulmonary valve replacement. During follow-up periods, functional class, residual RVOT stenosis and pulmonary regurgitation, tricuspid regurgitation, occurrence of ventricular and atrial arrhythmias were comparable between two groups. Conclusion: In adult ToF patients with severe preoperative cyanosis, more aggressive RVOT reconstruction and careful postoperative care are mandatory. However intermediate-term outcome of this group of patients is comparable to the patients with minimal or no preoperative cyanosis.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.976-981
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• 2007

Purpose : Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. Methods : We investigated 130 repaired TOF patients more than 15 years of age who followed-up by echocardiography from January 2002 to December 2003. Of 130 patients, we identified 17 patients with AoRo dilatation, which was defined as ratio of expected AoRo size by standard nomogram (AoRo ratio) >1.5 (dilator group), and 113 TOF controls, with AoRo ratio <1.5 (non-dilator group). Results : Mean indexed AoRo size ($mm/m^2$) in the first echo was $24{\pm}3.2$ in the dilator group and $18{\pm}3.4$ in the non-dilator group (P<0.0001). AoRo rate of change (mm/year) from the first to latest echo study was $1.6{\pm}3.8$ in dilator group and $0.05{\pm}1.6$ in the non dilator group (P=0.0021). Patients from the dilator group showed a higher prevalence of pulmonary atresia (P=0.031) and a history of aortopulmonary shunt before repair (P=0.048), moderate to severe AR (P=0.0065), and increased left ventricular end-diastolic dimensions (P=0.003). Conclusions : A subset of patients late after TOF repair may show progressive dilatation of AoRo. To identify and prevent long-term sequelae in this patient group, regular follow-up and speculation about AoRo after TOF repair is recommended.