• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.1
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• pp.21-30
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• 2005

Purpose: The aim of this study was to identify the clinical features and natural history of dietary protein induced proctocolitis (DPIPC) and to detect the causative foods of DPIPC, and to evaluate the effect of elimination of the foods on the course of the disease. Methods: Between March 2003 and July 2004, data from 30 consecutive patients with DPIPC who were followed for over 6 months, was reviewed. The diagnostic criterion used for DPIPC was an increase in the number of eosinophils in the lamina propria (${\geq}60per$ 10 high-power fields). In breast feeding mothers, 5 highly allergenic foods were eliminated from the maternal diet for 7 days, namely, allergenic food groups such as dairy products, eggs, nuts and soybean, fish and shellfish, and wheat and buckwheat. We observed the disappearance or appearance of hematochezia after elimination or challenge with the offending foods. Results: Before diagnosis infants were breast-fed (93.3%) or formula-fed (6.7%). Mean age at symptom onset was $11.5{\pm}5.1$ (5~24) weeks, and mean age at diagnosis was $17.8{\pm}9.5$ (8~56) weeks. Duration from symptom onset to diagnosis was $6.3{\pm}6.7$ (0~36) weeks. Mean peripheral blood eosinophil count was $478{\pm}320$ (40~1,790)/$mm^3$ and eosinophilia (> $250/mm^3$) was observedin 90.0% of patients. None of patients were found to have an increased serum IgE level. Of 15 patients that received sigmoidoscopy, nodular hyperplasia with erosion was observed in 93.3%. Of 27 patients whose mother ate the diet eliminated the 5 food groups, hematochezia diappeared in 74.1% of patients. Offending foods were identified as dairy products (37.5%), wheat and buckwheat (27.5%), fish and shellfish (20.0%), nuts and soybean (7.5%) and eggs (7.5%). A free maternal diet without patient's clinical symptoms was achieved at $29.4{\pm}8.7$ (9~44) weeks of patient's age, and a free baby diet without blood in stools was achieved at $37.5{\pm}9.7$ (12~56) weeks of age. Conclusion: DPIPC commonly occurs in exclusively breast-fed babies. Elimination of the above-mentioned 5 hyper-allergenic food groups from the maternal diet for 7days enables the detection of the offending foods. DPIPC is a transient disorder and 96.0% of patients can tolerate the offending foods at 12 months of age.

• Tuberculosis and Respiratory Diseases
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• v.44 no.1
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• pp.25-43
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• 1997

Introduction : The outcome and incidence of acute respiratory distress syndrome (ARDS) could be variable related to the varied definitions used for ARDS by researchers. The purpose of the national survey was to define the risk factors of ARDS and investigate the prognostic indicies related to mortality of ARDS in Korea according to the definition of ARDS determined by the American-European Concensus Conference on 1992 year. Methods : A Multicenter registry of 48 University or University-affliated hospital and 18 general hospital s equipped with more than 400 patient's beds conducted over 13 months of patients with acute respiratory distress syndrome using the same registry protocol. Results : 1. In the 12 months of the registry, 167 patients were enrolled at the 24 hospitals. 2. The mean age was 56.5 years (${\pm}17.2$ years) and there was a 1.9:1 ratio of males to females. 3. Sepsis was the most common risk factors (78.1%), followed by aspiration (16.6%), trauma (11.6%), and shock (8.5%). 4 The overall mortality rate was 71.9%. The mean duration was 11 days (${\pm}13.1$ days) from the diagnosis of ARDS to the death. Respiratory insufficiency appeared to be a major cause in 43.7% of the deaths followed by sepsis (36.1%), heart failure (7.6%) and hepatic failure (6.7%). 5. There were no significant differences in mortality based on sex or age. No significant difference in mortality in infectious versus noninfectious causes of ARDS was found. 6. There were significant differences in the pulse rate, platelet numbers, serum albumin and glucose levels, the amounts of 24 hour urine, arterial pH, $Pa0_2$, $PaCO_2$, $Sa0_2$, alveolar-arterial oxygen differences, $PaO_2/FIO_2$, and PEEP/$FI0_2$ between the survivors and the deaths on study days 1 through 6 of the first week after enrollment. 7. The survivors had significantly less organ failure and lower APACHE III scores at the time of diagnosis of ARDS (P<0.05). 8. The numbers of organ failure (odd ratio 1.95, 95% confidence intervals:1.05-3.61, P=0.03) and the score of APACHE III (odd ratio 1.59, 95% confidence interval:1.01-2.50, P=0.04) appeared to be independent risk factors of the mortality in the patients with ARDS. Conclusions : The mortality was 71.9% of total 167 patients in this investigation using the definition of American-European Consensus Conference on 1992 year, and the respiratory insufficiency was the leading cause of the death. In addition, the numbers of organ failure and the score of APACHE III at the time of diagnosis of ARDS appeared to be independent risk factors of the mortality in the patients with ARDS.

• Journal of Oral Medicine and Pain
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• v.34 no.1
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• pp.91-102
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• 2009

Condylar resorption, or condylysis can be defined as progressive alteration of condylar shape and decrease in mass. Condylar resorption is a poorly understood progressive disease that affects the TMJ and that can result in malocclusion, facial disfigurement, TMJ dysfunction, and pain. The aim of this study was to investigate clinical assessment and cephalometric characteristics in 224 patients with condylar resorption, who visited in the Department of Oral Medicine Kyungpook National University Hospital at 2006, by use of panorama, transcranial view and lateral cephalometric radiograph. The results were as follows; 1. Clinical assessment 1) Total number of patients who visited with chief complaints of TMD were 2419 and 224 (9.3%) among them revealed the condylar resorption, Among patients group with condylar resorption, female was 183 and male was 41, females were predominant. 2) Patient's age ranged from 12 to 70 and mean age was 30.6 years old with a strong predominance for 10s and 20s. Distribution of a showed as follows; 10s was 26.3%, 20s was 34,8%, 30s was 13.8%, 40s was 11.2%, 50s was 7.1%, 60s was 6.3% and 70s was 0.4%. 3) Most of the patients had parafunctional habit. 4) The case of showing the pain in condylar resorption was 145, the case of not showing the pain was 79. 5) Treatment duration of the patients was relatively short. 2. Cephalometric Characteristics 1) ANB which means the retruding of the mandible increased significantly than normal group. The ANB of female was lager than male group as the means of ANB were 5.05 in female and 3.57 in male, 2) SN-GoMe and FMA increased in resorption patients, but FH-PP did not show any significant difference. The FMA of female was lager than male group as the means were 31.69 in female and 30.44 in male. 3) Total posterior facial height was significantly smaller and total anterior facial height showed no significant increase as compared with those of the normal group. Condylar resorption was predominant in young female which was caused by more vertical facial pattern in female than male and increase of parafunctional habit in young age. It was thought that the patients who have a risk factor increasing the compressive stress at condyle caused by obliquely inclined masseter and medial pterygoid show high prevalence of condylar resorption.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.25-32
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• 2000

Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

• Journal of Chest Surgery
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• v.38 no.7 s.252
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• pp.489-495
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• 2005

In the surgical treatment of aortic dissection, aortic arch replacement under total circulatory arrest is often performed after careful inspection to determine the severity of disease progression. Under circulatory arrest, antegrade or retrograde cerebral perfusion is required for brain protection. Recently, antegrade cerebral perfusion has been used more, because of the limitation of retrograde cerebral perfusion. This study is to compare these two methods especially in the respect to neurological complications. Material and Method: Forty patients with aortic dissection involving aortic arch from May 2000 to May 2004 were enrolled in this study, and the methods of operation, clinical recovery, and neurological complications were retrospectively reviewed. Result: In the ACP (antegrade cerebral perfusion) group, axillary artery cannulation was performed in 10 out of 15 cases. In the RCP (retrograde cerebral perfusion) group, femoral artery Cannulation was performed in 24 out of 25 cases. The average esophageal and rectal temperature under total circulatory arrest was $17.2^{\circ}C\;and\;22.8^{\circ}C$ in the group A, and $16.0^{\circ}C\;and\;19.7^{\circ}C$ in the group B, respectively. Higher temperature in the ACP group may have brought the shorter operation and cardiopulmonary bypass time. However, the length of period for postoperative clinical recovery and admission duration did not show any statistically significant differences. Eleven out of the total 15 cases in the ACP group and thirteen out of the total 25 cases in the RCP group showed neurological complication but did not show statistically significant difference. In each group, there were 5 cases with permanent neurological complications. All 5 cases in the ACP group showed some improvements that enabled routine exercise. However all 5 cases in RCP group did not show significant improvements. Conclusion: The Antegrade, cerebral perfusion, which maintains orthordromic circulation, brings moderate degree of hypothermia and, therefore, shortens the operation time and cardiopulmonary bypass time. We concluded that Antegrade cerebral perfusion is safe and can be used widely under total circulatory arrest.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.1-11
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• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.12 no.1
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• pp.103-114
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• 2001

Tourette's disorder is a disease which manifests one or more motor tics and vocal tics for more than a year. Chronic motor tic or vocal tic disorders are characterized by only one kind of tics for more than a year. We intended to investigate the clinical characteristics of the patients with chronic motor tic disorders or Tourette's disorders who had admitted from May 1, 1998 to May 1, 1999 to Seoul National University Hospital Child and Adolescent Psychiatry ward. In addition, we compared the clinical characteristics of the patients in order to elucidate the relationship between the two disorders. The patients with learning disabilities were selected as controls. There was no statistically significant difference between the onsets of the patients with chronic motor tic disorders(n=13, $7.3{\pm}2.5$ years), and Tourette's disorder(n=39, $7.2{\pm}2.2$ years), but with learning disability($4.2{\pm}1.9$ years). Also, the patients with chronic motor tic disorder and Tourette's disorder showed similar age at admission($11.7{\pm}2.7$ versus $11.5{\pm}2.6$ years), duration of admission($5.7{\pm}5.4$ versus $11.0{\pm}8.7$ weeks), mothers' ages at child birth($27.3{\pm}2.9$ versus $28.3{\pm}6.7$ years old),and fathers' age at child birth($32.2{\pm}3.2$ versus $33.3{\pm}5.2$ years old). We observed that those who had learning disabilities were alike in those aspects, except for age at visit to clinic($9.8{\pm}3.2$ years old). Family history of psychiatric illnesses(24.1% versus 46.2%), recognized precipitating factors(11.1% versus 35.7%) and response to pharmacological treatments(77.8% versus 76.9%) of the patients with chronic motor tic disorders and Tourette's disorders were observed and no differences were found. Comorbid patterns of diseases were noted. Intrafamilial conflicts were more common in the patients with learning disabilities than those with chronic tic disorders or Tourette's disorders. Precipitating factors were observed more frequent in chronic tic disorder and Tourette's disorder than learning disability. Neurocognitive profiles were investigated, and verbal IQs of the patients with chronic motor tic disorder, Tourette's disorder and learning disability were $92.3{\pm}10.7$, $94.7{\pm}14.9$, $94.3{\pm}13.8$, performance IQs $93.0{\pm}20.5$, $97.5{\pm}13.0$, $95.0{\pm}16.9$ and full-scale IQs $91.9{\pm}20.1$, $95.8{\pm}14.5$, $93.9{\pm}15.1$, respectively, which were found to be not significantly different. No difference was found in structural neurological abnormalities and EEG profiles. The patients with learning disabilities showed more common Bender-Gestalt test abnormalities. In conclusion, we have not found any affirmative clues for the division of chronic motor tic disorder and Tourette's disorder in clinical perspective.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1204-1212
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• 2002

Purpose : Early surfactant therapy with either gentle ventilation, high-frequency ventilation or aggressive weaning of mechanical ventilation are principles for the treatment of respiratory distress syndrome(RDS). We studied to determine the accessibility of noninvasive nasal continuous positive airway pressure(CPAP) rather than mechanical ventilation by invasive intubation after early surfactant therapy. Methods : The study group consisted of 14 infants who were born and diagnosed with moderate respiratory distress syndrome and received early surfactant therapy with nasal CPAP of PEEP 5-6 cm $H_2O$ within two hours after birth in the Fatima neonatal intensive care unit for two years from January 1999 to August 2001. The control group consisted of 15 infants who were diagnosed with the disease and could be weaned from mechanical ventilator within five days after birth during the same period. Results : The characteristics, the severity of clinical symptoms and laboratory findings in the two groups at birth showed no significant difference. Neither did the interim analysis of laboratory data in two groups. Of 14 infants in the study group who received nasal CPAP after early surfactant therapy, only two infants showed weaning failure with this therapy. In the response cases, duration of CPAP was five days and mean airway pressure was $5.4{\pm}0.5cm$ $H_2O$. Two had the complication of CPAP with abdominal distension. Final complications and outcomes in the two groups showed no signifcant difference(P>0.05). Conclusion : The clinical courses in the two groups showed no significant difference. Therefore, we suggest that early surfactant therapy with noninvasive nasal CPAP is a simple and safe method rather than aggressive weaning after invasive mechanical ventilation in moderate respiratory distress syndrome.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.320-328
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• 2008

Background: Numerous surgical devices for mitral repair have been used in the past with good results. In this study we describe a simple annuloplasty technique with using a new device ($Mitracon^{(R)}$). The aim of this study was to assess its efficacy and surgical results with using $Mitracon^{(R)}$. Material and Method: From May 2003 to October 2005, 46 patients (21 women and 25 men (mean age of $51.4{\pm}17.8$ years) with mitral regurgitation from various causes were treated with either the $Mitracon^{(R)}$ (the $Mitracon^{(R)}$ group) or the Capentier Edward rigid ring (the CE group). The median follow-up duration was 18.9 months. Result: The mean grade of mitral regurgitation before and immediately after surgery in the $Mitracon^{(R)}$ group and the CE group decreased from $3.2{\pm}0.8$ to $0.6{\pm}0.7$ and $3.4{\pm}0.7$ to $0.3{\pm}0.5$, respectively. There were no significant changes in the ejection fraction either between the two groups or before and immediately after surgery. No deaths were seen in either group. Early postoperative echocardiography of all 46 patients showed only trivial mitral regurgitation or none at all. Echocardiography at a median of 18.9 months also showed no progression in mitral regurgitation. The mean grade of mitral regurgitation in the $Mitracon^{(R)}$ group at this time point decreased from $3.2{\pm}0.8$ to $0.8{\pm}0.7$ (p<0.05). The CE group also showed a similar degree of decrease from $3.4{\pm}0.7$ to $0.3{\pm}0.6$ (p<0.05). The mitral valve area in the $Mitracon^{(R)}$ group at 1 year follow-up was $3.3{\pm}0.9cm^2$. The mitral valve area in the CE group was $2.7{\pm}0.6cm^2$. The mean mitral pressure gradient in the $Mitracon^{(R)}$ group at 1 year follow-up was $3.1{\pm}1.3$ mmHg. The mean pressure gradient in the CE group was $4.5{\pm}2.1$ mmHg, although any statistical significant difference for this between the groups was not reached. Conclusion: The present study showed the described technique to be safe and effective in the intermediate term. Because long term results are unavailable, a more extensive prospective randomized multicenter trial may be warranted to determine whether this procedure should be generally applied for repair of mitral valve disease.