• Clinical and Experimental Pediatrics
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• v.50 no.1
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• pp.40-46
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• 2007

Purpose : This study assessed the long term survival rate and long term complications of patients who had a modified Fontan operation for functionally univentricular cardiac anomaly. Methods : Between June 1986 and December 2000, 302 patients with a functional single ventricle underwent surgical interventions and were followed up until February 2006. The mean follow-up period was $8.3{\pm}5.3years$ (range 3.5-18 years). Their median age was 2.4 years at the Fontan operation. The survival rate, the incidence and the risk factor of late complications were evaluated retrospectively. Results : The verall survival rate was 91 percent at 5 years and 87 percent at 10 years. In multivariate analysis, early calendar year of operation and significant regurgitation were risk factors of death. The surviving patients showed NYHA functional class I in 82 percent, class II in 15 percent, and class III in 3 percent. Redo Fontan operations were necessary in 8.8 percent of patients at average $12.8{\pm}3.6years$ after initial Fontan operation. The most common cause of Fontan conversion was atrial arrhythmia. The incidence of thromboembolic events was 9.3% and these complications were associated with the occurrence of atrial tachyarrhythmia. Supraventricular tachycardia including atrial flutter or fibrillation were reported on the follow-up examination by 11.2 percent of survivors after $8.4{\pm}5.6years$. Atriopulmonary connection showed higher rates of late tachycardia than lateral tunnel operation. Conclusions : This study revealed that the recent survival rate of Fontan type operation was satisfactory, but the occurrence of late complications after a Fontan type operation increased with the longer survival. There is a need for strict follow up and early treatment of late complications in patients who had a Fontan operation.

• Radiation Oncology Journal
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• v.10 no.1
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• pp.35-41
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• 1992

Forty eight patients with adenoid cystic carcinoma (ACC) in the head and neck treated between 1979 and 1990 were reviewed according to treatment modalities and clinical courses. The common site of origin was minor salivary gland ($69\%$), mostly hard palate and maxillary sinus. 11 patients received radiation therapy (RT) alone and 37 patients received combined surgery and radiation therapy. The follow-up period of the survivors ranged from 4 to 118 months (median 49.5 months). The 5 year local control rate was $69.3\%$, $67.3\%$ and 83.9$\%$ in RT alone, conservative operation (OP)+RT and radical OP+RT group, respectively (p>0.05). The control of local disease was best achieved with radical OP+RT. In postoperative RT, high dose ($\ge$60 Gy) and generous field size($\ge$64 cm$^2$) yielded a better local control rate. Actuarial overall survival rate was $79.0\%$, at 5 years and $19.2\%$ at 10 years. Distant metastases (DM) developed in $40\%$ of patients, mostly in the lung. Disease-free (NED) survival rate was $45.8\%$ at 5 years and significant differences did not exist according to primary sites and treatment options. Survival rate after the onset of DM was $19.5\%$ at 5 years. Occurrence of DM tends to lower survival significantly. In an effort to find the role of RT in the treatment of ACC, our data suggest that a well-planned postoperative RT employing a high dose and generous field size can produce high local control rate and remaining urgent problem of distant metastasis demands more effective chemotherapy for the purpose of improving survival of ACC patients.

• Neonatal Medicine
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• v.18 no.2
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• pp.288-292
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• 2011

Purpose: The incidence of methicillin-resistant Staphylococcus aureus (MRSA) infection has increased in children and in neonates, and is particularly associated with frequent use of central venous catheter in very low birth weight (VLBW) infants. It is known that the morbidity and mortality of MRSA infection are low in neonates, as compared with adults. The objective of this study was to examine the difference in clinical characteristics between VLBW infants that survived and those that did not, a catheter-related bloodstream infection (CRBSI) of MRSA. Methods: Thirty-four VLBW infants had laboratory-confirmed bloodstream infection with S. aureus. We examined the incidence, mortality and morbidity of CRBSI, and predictive factors associated with mortality. Results: Twenty-six infants had same pathogen (24 MRSA, 2 Methicillin-sensitive Staphylococcus aureus) in the blood and in the catheter tip. Eight infants (25.8%) died in the CRBSI and they all had MRSA blood infections. Sex ratio, gestational age, duration between blood collection and identification of pathogens, and WBC and platelet count were not significantly different between patients that died from and patients that survived CRBSI of MRSA. C-reactive protein (CRP) was significantly higher in VLBW infants that died. Mean age of onset and hospital day was earlier (9.1${\pm}$6.6 vs. 26.9${\pm}$20.2; P=0.005) and shorter for patients that died (10.1${\pm}$7.0 vs. 73.0${\pm}$32.4; P=0.000). Two survivors had complications of pyogenic arthritis of the lower extremities and soft tissue infection, respectively. Conclusion: Mortality of CRBSI was likely to be high in VLBW infants and might be anticipated by CRP and early onset of disease.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.523-528
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• 2005

Background: Investigation of the change of ventricular function and mitral regurgitation after surgical repair of patient with anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is key issue for the better surgical outcome. Material and Method: From April 1986 to July 2002, 12 patients presented with ALCAPA. The median age at repair was 4 months. Surgical methods included left coronary artery transfer to the aorta (10), Takeuchi procedure (1), saphenous vein free graft bypass (1). Mitral valve was repaired in 1. Result: There were 2 hospital death $(16.7\%)$. The mean follow-up period was $7.1\pm4.1$ years (range, 7 months to 13 years). Four patients required postoperative circulatory assist for $2.2\pm1.1$ days and one needed left ventricular assist device (LVAD) for 1day. Postoperative echocardiography demonstrated significant improvements in mean fractional shortening $(33.4\pm9.1\%\;vs\;17.7\pm9.6\%,\;n=10,\;p<0.05);$ left ventricular end diastolic dimension $(33.4\pm7.3\;mm\;vs\;44.8\pm7.0\;mm,\;n=10,\;p<0.05)$ and systolic dimension $(22.2\pm7.5\;mm\;vs\;33.4\pm7.9\;mm,\;n=10,\;p<0.05)$. Severities of mitral regurgitation decreased in all survivors at 1st and 4th year follow-up echocardiography. There were 2 reoperation due to residual MR and right ventricular outflow obstruction (Takeuchi case). Conclusion: Anatomic repair of anomalous left coronary artery from the pulmonary artery offered an excellent surgical results, especially in terms of the recovery of left ventricle function and mitral regurgitation. However, preoperative indications for mitral procedure is to be evaluated.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.675-679
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• 2005

Background: Clinical reports on replacement of all three (AV + MV + TV) valves are rare. Material and Method: From January 1992 to December 2003, 38 patients received triple valve replacement (aortic, mitral, tricuspid) at Yonsei Cardiovascular Center. Mean age of patients was $49.5\pm10.7 (28\~69)$ years, and 24 patients $(63.1\%)$ were female. Rheumatic valve disease was the most common cause of operation (n=37). Preoperative New York Heart Association functional class were II in 4, III in 24 and IV in 10. Fifteen patients (group 1) received triple valve replacement at their first operation. Twenty three patients (group 2) received one or more operations before tricuspid valve replacement (TVR). Seven patients received tricuspid valve annuloplasty at first operation and received TVR later. Result: Six patients died at hospital after operation $(15.8\%)$ and all these patients were in group 2. All patients in group I survived and were discharged. Three patients $(9.4\%)$ died during follow up periods. Most of the survivors had improved functional class (I in 22, II in 8, III in 1, IV in 1). During follow up period, there were 4 valve related complications. The 10-year survival rate was $68.8\%$ and survival rate for free from valve related event at 10 years was $85.5\%$. Conclusion: After triple valve replacement, most patients showed improvement of symptoms. And during follow up period, valve related complications and survival were acceptable. Therefore, if indicated, triple valve replacement is recommended before the patients' conditions get worse.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.854-861
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• 2002

In this study, we reviewed our early and long-term surgical results of complete atrioventricular septal defect during the last 16 years at our hospital. Materials and Methods: Between April 1986 and March 2002, 73 patients with complete atrioventricular septal defect underwent total correction without preceding palliation. Age at repair ranged from 2 to 85 (median age, 7) months, and weight ranged from 3 to 22 (median weight, 5.9)kg. Follow-up was complete with a mean duration of 69$\pm$51 months. Results: Overall operative mortality was 16.4%(12) with 3 late deaths. One, 5, and 10 year actuarial survival rates were 96.3 %, 94.2 % and 94.2 % respectively Sixteen of 61 (22.2%) operative survivors have undergone reoperation for postoperative mitral regurgitation or left ventricular outflow tract obstruction (LVOTO). Freedom from mitral reoperation at 1, 5, and 10 years were 87.8 %, 72.4 %, and 57.8 % and freedom from LVOTO at 1, 5, and 10 years were 98.2 %, 86.3 %, and 83.2 % respectively. Summary: In this study, we found that our early surgical results improved with quite an acceptable long-term outcome. Close observation of remaining mitral regurgitation was necessary, A precise evaluation of the atrioventricular valve morphology, a meticulous surgical technique, and the adequate postoperative management are mandatory for the excellent results.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.727-734
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• 2004

The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.862-870
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• 2002

The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.

• Journal of Chest Surgery
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• v.36 no.12
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• pp.961-969
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• 2003

The purpose of this study was to evaluate the fate of left atrioventricular valve regurgitation(LAVVR) following repair of complete atrioventricular septal defects (AVSDs). Material and Method: Between July 1984 and March 2002, repair of complete AV defects were performed in 77 patients. Mean age at surgery was 30.23$\pm$69.11 months (range 1 to 456). Echocardiograms of all survivors after isolated AVSDs correction were reviewed. LAVVR were evaluated with color doppler echocardiography in 64 survival periodically. On each study, LAVVR severity was graded on a 1 to 4 scale, based upon the size of the regurgitated jet. Result: Mild deterioration of LAVV function was fairly common. LAVVR severity increased by >1 grade in 19 patients (30.2%) during the course of the study. However, the deterioration in LAVVR function occurred primarily between 12 and 24 months postoperatively. After the initial 24 postoperative months, LAVVR worsened on only 8 occasions and in each instance worsened by only 1 grade. Deterioration more than 3+ LAVVR occurred in only 3 patients. And deterioration to 4+ LAVVR was not observed after the initial 24 postoperative months but one. Survival curve analysis predicted a 88.2% of ten-year freedom rate from development of 4+ LAVVR after initial operation of complete AVSDs. Conclusion: Postoperative LAVVR remains fairly stable following AVSDs repair, Serious deterioration is rare after 24 postoperative months, especially after the initial 48 postoperative months. But serial follow-up study with echocariogram was need till 24 postoperative months after repair of complete AVSDs.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.299-304
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• 2009

Background: Although the results of the surgical management for complete atrioventricular septal defect (c-AVSD) have improved, the optimal surgical strategy is still controversial. The aims of this study are to evaluate the outcome of c-AVSD repair and to define the risk factors related to reoperation. Material and Method: We retrospectively reviewed the medical records of 35 patients (8 males and 27 females) who underwent the total correction of c-AVSD from August 1996 to March 2008. The median age at repair was 5.2 months (range: 3 days$\sim$82 months). Sixteen patients (45.7%) were associated with Down syndrome. Prior palliative operations were performed in 4 patients. The one-patch techniques were performed in 3 patients, and the two-patch techniques were done in 32 patients. Result: There was 1 early death (2.9%). The median follow-up period was 68 months (range: $2\sim134$ months) for 34 survivors. There was no late death. Reoperations were performed in 5 patients (14.3%) for severe left atrioventricular valvular regurgitation (AVVR). Nine patients (25.7%) showed left an AVVR of more than grade III. Associated major cardiac anomalies and the use of Gore-Tex patch for ventricular septal closure were the risk factors for postoperative left atrioventricular valve failure and reoperation. Conclusion: In this study, we found that surgical repair of c-AVSD was safe and effective. However, the high reoperation rate after repair remains a problem to be solved.