• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.167-172
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• 2003

Purpose : Pulmonary vascular hypertension is a common problem in congenital heart disease, the most common cardiac condition in childhood. However, the mechanisms responsible for this pathologic change, treatment, and prevention are poorly understood. Therefore, we studied the gene expression of vascular endothelial growth factor(VEGF) by using a hypoxic model of the pulmonary artery smooth muscle cells. Methods : The main pulmonary artery and its proximal branches of a 6 wk old Fischer rat were excised. They were cut into multiple small pieces and suspended in DMEM medium supplemented with 20% fetal bovine serum and incubated in 5% $CO_2$-95% air atmosphere. The smooth muscle cells were confirmed by immunostaining with smooth muscle myosin and ${\alpha}$-smooth muscle actin antibodies. The VEGF gene expression in the hypoxic group was compared with the one in control the group as well as the one in the starved group by RT-PCR and Northern blot hybridization. Results : There was no statistically significant difference among the control, hypoxic and starved groups. Conclusion : There are few studies of pulmonary vascular hypertension at the molecular level in Korea. Therefore, we studied the expression of VEGF gene in hypoxic pulmonary vascular smooth muscle cells. Further studies will be needed to find the difference between newly born and adult rats, or human and rat pulmonary vascular smooth muscle cells in gene expression. We hope that the study will lead to a better understanding of pulmonary vascular hypertension.

• Journal of Chest Surgery
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• v.29 no.12
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• pp.1354-1359
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• 1996

Few observation have been made on the pericardial pressure and little is known about the composition of he pericardial fluid. So we studied the basic qualitative and quantitative analysis of the pericardial fluid in the patients with cardiac disease either congenital heart diasese(group A) or acquired heart disease(group B). The pressure of the pericardial cavity was measured by the method of open tipped water filled small polyethylene catheter connecting to the standardized monitor, which was introduced into pericardium of the patients who were performed pericardial incision for the heart or pericardial surgery. All of the data was compared to the simultaneously checked hematologic value of the same patient. The mean pressure of the pericardial cavity was 2.4mmHg and the amount of the pericardial fluid was 13cc/m2 of body surface for the group A and 17.7cc for the group B. And the cell count was 138$\pm$l16/1 in group A and 230$\pm$135/1 in group B and the pH was 7.83$\pm$0.40 in group A. 7.80$\pm$0.52 in group B. Pericardial fluid revealed satisfactically significant alkaline pH than plasma. The fundamental electrolyte, Wa+, K+, Cl and glucose were identical to the hematologic values of the same patient, but the protein concentration was 2.Bg/dL for group A and 3.Ig/dL for group B heart disease and those were remarkable low concentration compared to the hematologic value of the same patient. LDH and amylase were identical to the value of the serum of the same patient, but the concentration of LDH of group B was slightly higher than that of the group A.

• Journal of Preventive Medicine and Public Health
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• v.34 no.4
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• pp.427-436
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• 2001

Objective : To estimate the prevalence of congenital heart disease from the 1990 student heart disease screening program. Methods : The heart disease screening program for elementary students was conducted in Kyonggi-do, in 1998. The subjects of the present study comprised the 40,402 students who attended the schools in the catchment area of a collaborative university hospital and who participated in the primary examination. The congenital heart disease (CHD) patients were initially identified through a questionnaire about prior medical history, and further through diagnostic tests & medical examinations in the secondary & the tertiary examinations. Certain assumptions were used in the estimation of the number of CHD cases among non-participants of the secondary & tertiary examinations. The overall prevalence of CHD was estimated by adding the CHD detection rates of the participants and the estimated prevalence of the non-participants. Results : Among the 40,402 primary participants, 1,655 were referred further, of whom 79.1% (1,309) participated in the secondary examination. Of these, 121 were referred to the tertiary examination, with a participation rate at this last stage of 80.2%. The positive predictive value (PPV) of the screening tools was the highest when the results of both EKG and the questionnaire were positive. Because 85.9% of the detected cases had a past history of CHD, PPV was higher when the selection criteria in the questionnaire included past CHD history than when it didnt. The CHD defection rate among the participants was 1.76 cases/1,000 and the presumed number of cases among the non-participants was 31; giving an estimated final CHD prevalence of 2.52 cases/1,000 (95% CI : 2.06-3.06). Among the identified cases of CHD, VSD (52.8%) was the most common, followed by PDA (9.7%), TOF (9.7%) & PS (9.7%). Conclusion : Because the characteristics of the non-participants differed from those of the participants, the estimation of prevalence was influenced by the participation rate. Of the detected cases, 85.9% had a past history of diagnosis or operation for CMD. These findings suggested that the prevalence estimated in this study may be an underestimation of the actual condition. Therefore, a birth cohort study is required in order to more accurately estimate the prevalence and the effects of the program.

• Journal of Chest Surgery
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• v.34 no.11
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• pp.831-842
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• 2001

Background: Patients with cardiac diseases who have structural defects in their heart bring about metabolic insult such as preoperative acid-base imbalance. Cardiac operation requires many nonphysiologic procedures such as extracorporeal circulation, hypothermia, and hemodilution. We studied the acid-base status of surgical heart diseases pre-operatively, during extracorporeal circulation, and post-operatively and researched the treatment indications of acid-base disturbances. Material and Method: From January 1997 to May 1999, fifty two cases of open heart surgery were carried out under extracorporeal circulation, which divided into a set of pediatric and adult groups, congenital and acquired groups, non-cyanotic and cyanotic groups, The $\alpha$ -stat arterial blood gas analysis was done in each group during the preoperative period, during the operation with extracorporeal circulation, and during the postoperative period. Result: Before surgery, all patients present metabolic acidosis, PaO2 was low in adult group and acquired group and compensatory respiratory alkalosis was noted in cyanotic group. During extracorporeal circulation, adult group revealed alkalosis and normal in acquired group. Pediatric group presents low Pa$CO_2$, metabolic acidosis and respiratory alkalosis. Congenital group and non-cyanotic group showed non-compensatory alkalosis trend and non-compensatory respiratory acidosis were observed in cyanotic group during extracorporeal circulation. Postoperative acid-base status of adult group was recovered to normal and the standard bicarbonate was increased in the acquired group. All of the pediatric, congenital non-cyanotic, and cyanotic groups revealed the lack of buffer base.

• Journal of Chest Surgery
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• v.35 no.7
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• pp.517-522
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• 2002

Background: Congenital heart surgery may lead to myocardial swelling and hemodynamic instability. Delayed sternal closure may be beneficial in this setting. The purpose of this study was to assess mortality and mediastinal infection rate associated with delayed sternal closure after congenital heart surgery and to evaluate the risk factors which affect mortality and mediastinal infection rate. Material and Method: We retrospectively reviewed 40 patients who underwent delayed sternal closure after repair of congenital heart disease at Yonsei Cardiovascular Hospital, from January 1994 to May 2001. In these patients, we assessed the mortality and mediastinal infection rate, and evaluated their risk factors including operation time, bypass time, aortic cross clamp time, duration to sternal closure and postoperative artificial ventilation time. Mediastinal infection was defined to have positive culture in mediastinum. Result: Hemodynamic instability was the most common indication for delayed sternal closure(n=36) and other indications included postoperative bleeding(n=2) and conduit compression(n=2). The median age at operation was $14.4{\pm}33.4$months old(range, 2days-12years). The patients with postoperative bleeding and conduit compression were much older than the others. The sternum was left open for $4.5{\pm}3.4$ days(range, 1-20days). Overall mortality was 25%(10/40) and mediastinal infection occured in 24.3%(9/37) (3 patients were excluded in mediastinal infection for early death). In risk factor analyses, only aortic cross clamp time had statistical significance for mortality in univariate analyses. However, multivariate analyses revealed that there were no significant predictors for risk of mortality and mediastinal infection. Conclusion: Delayed sternal closure after repair of congenital cardiac disease had relatively high mortality and mediastinal infection rate. But, in patients with hemodynamic instability, postoperative bleeding and conduit compression after repair of congenital cardiac disease, delayed sternal closure may be an effective life saving method.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.363-365
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• 2008

Accessory mitral valve tissue is a very rare congenital cardiac malformation and it is an uncommon cause of left Ventricular outflow tract obstruction(LVOTO). The pathogenetic mechanism of subaortic obstruction is thought to be systolic ballooning of the abnormal valve tissue into LVOT. We are reporting a case of an accessory mitral valve tissue that was associated with LVOTO, and this was completely relieved after trans-aortic surgical excision of the accessory tissue.

• Journal of Veterinary Clinics
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• v.27 no.6
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• pp.735-739
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• 2010

An 8 month old female Maltese (body weight 3.6 kg) was referred with primary complaints of dyspnea and exercise intolerance. Diagnostic imaging studies revealed marked cardiomegaly and prominent main pulmonary trunk dilation on thoracic radiography, abnormally arisen aortic roots (toward right ventricle) with left-to right shunted perimembraneous ventricular septal defect located underneath of aortic root, aortic root was located to predominantly to the right ventricle and pulmonary regurgitation (peak velocity 4.7 m/s, pressure gradient ~88 mmHg) from pulmonary over-circulation and hypertension on echocardiography, indicating double outlet right ventricle (DORV). The dog was treated with furosemide (1 mg/kg, BID) for reducing volume overload at right ventricle, spironolatcone (1 mg/kg) and enalapril (0.5 mg/kg) for minimizing deleterious cardiac remodeling, and sildenafil (1 mg/kg) for lessening pulmonary over-circulation and hypertension. The clinical condition of this dog was improved after 1 week of medical treatment. The dog is currently survived and regularly monitored.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.796-799
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• 2004

A 52 day-old male infant who had Taussig-Bing anomaly with coarctation of the aorta underwent initial palliative Damus-Kaye-Stansel (DKS) procedure including arch reconstruction because of suspected intramural coronary artery, size discrepancy of great arteries, potential subaortic stenosis, refractory pneumonia, and severe congestive heart failure. Total repair was done 44 months later, which was composed of VSD patch closure, DKS take-down, and arterial switch procedure, We report a successful case of DKS take-down and arterial switch operation for the reuse of native aortic and pulmonary valves rather than Rastelli-type procedure in a patient with Taussig-Bing anomaly having palliative DKS procedure.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.633-636
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• 2005

Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacemcent of tricuspid valve mechanism. We elected to perform modified Starness operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.