No abstract available.
Catheters* ; Heart Defects, Congenital*

Double outlet right ventricle, a complex of congenital cardiac anomalies in which both great arteries arise wholly or in large part from the morphologic right ventricle presents diverse clinical manifestations according to the morphological characteristics. Eighty cases with DORV were diagnosed and operated at Yonsei Cardiovascular Center from 1988 to 1992. The analysis of the morphological characteristics of this anomaly and comparative study of operative methods and mortality according to the morphological classification led to the following results: 1) The location of VSD varied to be subaortic in 40 cases, subpulmonic in 15, doubly committed in 6 and noncommitted in 19 cases. The great arteries were interrelated in D-malposition in 43 cases and L-malposition in 18 and 26 cases among above mentioned 61 cases revealed the side-by-side relationship. Ten of the cases showed normal position and the rest 9, A-malposition. 2) Pulmonary stenosis was found in 60 cases, and when associated with subpulmonic VSD, occurring less frequently. PDA and ASD secundum were associated in successional order and only 3 cases were verified to carry aortic arch anomalies such as coarctation and interruption, all with subpulmonic VSD. 3) Intraventricular tunnel repair was possible in every case associated with subaortic VSD and the postoperative prognosis was excellent. In comparison, most of the cases associated with other types of VSD in which only palliative surgery or various types of intraventricular repair were performed, the general outcome was poor. But total cavopulmonary connection, one of the physiological corrective methods, showing a better postoperative prognosis is being carefully considered for its broad adoptation in future. In conclusion, double outlet right ventricle, a pathophysiological complex of various anomalies, should be thoroughly evaluated for the morphological characteristics to decide the most appropriate types of operation and for consequent improvement of prognosis.
Aorta, Thoracic ; Arteries ; Classification ; Double Outlet Right Ventricle* ; Heart Ventricles ; Mortality ; Palliative Care ; Prognosis ; Pulmonary Valve Stenosis

PURPOSE: Cytogenetic and genetic alterations of tumors are closely related with progressian and promotion of cancers. Comparative genomic hybridization (CGH) has known to be a novel tool for the detection of genetic alteration in solid cancers. We performed CGH for the detection of new genetic alterations of bladder tumors. MATERIALS AND METHODS: Biotin-labeled tumor DNA and digoxigenin-labeled normal DNA were hybridized to normal metaphase cells. The fluorescence signals were captured by fluorescence microscope after detection by avidin FITC and antidigoxigenin rhodamin. Then, the ratio of fluorescence was calculated by an image analyzer. RESULTS: CGH results showed amplifications on chromosomes 1q, 3q, 4q, 5p, 6pq, 7p, 8q, 11q, 12q, 13q, 17q, 18q and 20pq (more than 20% of cases). Deletions were on chromosome 2q21-qter, 4q13-q23, 5q, 8p12-p22, 9pq, 11p13-p15 (more than 20% of cases). High level amplifications were noted on chromosomes 1q31-qter, 3p21, 3q24, 4q26, Sq21-qter, llq14-qter, 12q15-q21, 12q21-q24, 13q21-q31, 17q22, 18q22. CONCLUSION: We considered that the amplification on chromosome 4q26, 11q14-qter, 12q21-q24, 18q12 and deletion on 4qll-4q13 as a novel genetic alterations of bladder cancer. Our results revealed different pattem of amplifications that affect other regions from previous study on chromosome 7, llq, 12q, 13q, and 18q. CGH was very useful for the screening of genetic alterations of solid tumors.
Avidin ; Chromosomes, Human, Pair 7 ; Comparative Genomic Hybridization* ; Cytogenetics ; DNA ; Fluorescein-5-isothiocyanate ; Fluorescence ; Mass Screening ; Metaphase ; Urinary Bladder Neoplasms* ; Urinary Bladder*

PURPOSE: The present study was designed to evaluate the incidence and characteristics of acquired cystic kidney disease (ACKD) in patients with end-stage renal disease who were treated by chronic hemodialysis, using renal ultrasonography. MATERIALS AND METHODS: We performed renal ultrasonography as a screening modality in 112 patients treated with long-term hemodialysis due to end-stage renal disease. We classified the patients years from one to six years. RESULTS: In our study, cystic lesions were noted in 41 patients (36.6%) and there was no sexual predilection of ACKD. Also, a prolonged period of hemodialysis increased the incidence of ACKD, especially for those treated more than 49 months. We encountered a case of renal cell cancer, evaluated it with CT, and then treated it by radical nephrectomy. CONCLUSIONS: With these results, renal ultrasonography can be used as a screening modality in patients treated with long-term hemodialysis more than 49 months due to end-stage renal disease. As complications occur, further evaluation will be needed.
Carcinoma, Renal Cell ; Humans ; Incidence ; Kidney Diseases, Cystic* ; Kidney Failure, Chronic ; Mass Screening ; Nephrectomy ; Renal Dialysis* ; Ultrasonography

Augmentation cystoplasty is effective in the rehabilitation of the contracted bladder of small capacity caused by tuberculosis or interstitial cystitis refractory to more conservative methods of management. Genitourinary tuberculosis is the most common cause of contracted bladder in Korea and it may lead to considerable vesicoureteral reflux and progressive deterioration of renal function. The use of hemi-Kock ileal pouch with an afferent intussuscepted nipple is a suitable solution in patient in whom the bladder outflow tract provides adequate resistance and supratrigonal cystectomy is to be performed with construction of an antireflux mechanism. Herein, we report 4 cases of hemi-Kock ileocystoplasty.
Cystectomy ; Cystitis, Interstitial ; Humans ; Korea ; Nipples ; Rehabilitation ; Tuberculosis ; Urinary Bladder ; Vesico-Ureteral Reflux

The hemodynamic feature of the tetralogy of Fallot (TOF) is best characterized by equality of left and right ventricular peak systolic pressure. The interventricular septum is flattened and thickened. Even after the right ventricular pressure is normalized in the postoperative period, the morphological abnormality continues for some time We have analyzed the morphological changes of the IVS echocardiographically after total correction and tried to correlate them with the prognosis of the patients. Forty five patients underwent total correction of TOF between May 1991 and July 1992.The IVS morphology was assessed with 2-D echocardiography before and after surgery. The control group consisted of 15 normal subjects(8 with functional cardiac murmur and 7 with non-cardiac diseases).We also evaluated the influence of the residual pulmonary regurgitation(PR) or pulmonic stenosis (PS) upon sequential changes in the end-systolic configuration and the thickness of the IVS. The following results were obtained. 1) Four months after total correction the IVS thickness and configuration at end-systole of the study patients were normalized, and they were not significantly different compared to those of the control group. 2) The postoperative residual PR of mild to moderate degree did not influence the course of morphological normalization. On the other hand, the postoperative residual PS greater than moderate degree hindered the normalization process. And the morphological abnormalities in the patients with the residual PS lasted longer than in the patients with the residual PR. 3) It would be necessary to perform cardiac catheterization in order to evaluate and to take care of the residual hemodynamic abnormalities if the IVS morphology remains to be abnormal even after one year after total correction.
Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Echocardiography* ; Hand ; Heart Murmurs ; Hemodynamics ; Humans ; Postoperative Period ; Prognosis ; Pulmonary Valve Stenosis ; Tetralogy of Fallot* ; Ventricular Pressure

PURPOSE: Type III 5-alpha reductase (SRD5A3; steroid 5-alpha reductase 3) may be associated with the progression of prostate cancer (PCa). The aim of our study was to determine whether the length of AC repeats in the SRD5A3 gene is associated with the risk of PCa and the expression of androgen receptor (AR) protein in Korean men. MATERIALS AND METHODS: We compared the length of AC repeats in the short tandem repeat (STR) region of the SRD5A3 gene in 68 PCa patients and 81 control subjects by genotyping. A total of 55 patients in the PCa group underwent radical prostatectomy. We evaluated the expression of AR protein by using Western blotting and tested the association between the type of AC repeats in the SRD5A3 gene and AR protein expression and clinical and pathologic parameters. RESULTS: The short type of STR had less than 21 copies of AC repeats in the SRD5A3 gene. The SS type (short and short type) of STR of the SRD5A3 gene was 2.2 times as likely to occur in PCa patients as in controls (odds ratio, 2.21; 95% confidence interval, 1.14 to 4.31; p=0.019). However, AC repeats of the SRD5A3 gene were not associated with AR protein expression or clinical or pathologic parameters in PCa samples. CONCLUSIONS: These results suggest that the short AC repeats of SRD5A3 polymorphism are associated with an increased risk of PCa. SRD5A3 polymorphism may contribute to a genetic predisposition for PCa.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase ; Blotting, Western ; Coat Protein Complex I ; Genetic Predisposition to Disease ; Humans ; Microsatellite Repeats ; Oxidoreductases ; Passive Cutaneous Anaphylaxis ; Polymorphism, Genetic ; Prostate ; Prostatectomy ; Prostatic Neoplasms ; Receptors, Androgen

Retroperitoneal teratoma is a rare congenital neoplasm derived from three germ layers and generally recognized as a disease of childhood. Those who were not operated on did not fare any better. The mortality from the disease was virtually 100%. Recently, we experienced a case of retroperitoneal teratoma.
Germ Layers ; Mortality ; Teratoma*

BACKGROUND: The hemodynamic superiority of total cavopulmonary anastomosis (TCPA) over atriopulmonary connection as a modification of Fontan-type operation, began to be acknowledged with more applications to clinical cases. The need of adequate resolutions for the investigation and improvement of residual hemodynamic derangements including abnormal distribution of the pulmonary blood flow, is emerging. METHODS: We studied 20 patients (M:F=12:8, age:67.9+/-41.5 months) who have had the TCPA and were followed-up by cardiac catheterization, angiography and lung perfusion scan 24.5+/-15.7 months after the operation. Pulmonary arterial growth and hemodynamic influences including the pulmonary blood flow distribution were investigated to verify the appropriateness of the conventional TCPA method and to aid in the determination of the consequences and prognosis of the operation. RESULTS: The mean pulmonary arterial pressure before and after the operation was 15.1+/-3.2 mmHg and 13.9+/-4.8 mmHg respectively without significant difference and there was no significant postoperative changes in the cross-sectional area index of pulmonary artery in regard to the variability of body surface area. The pulmonary blood flow was distributed with a greater amount in the ipsilateral side of IVC flow entrance (IVCipsi) than the contralateral side (IVCcontra), with an ipsilateral to contralateral perfusion ratio (i/cPR) of 1.24+/-0.42. Comparing the subgroups by the type of superior vena caval inflow, unilateral superior cavopulmonary anastomosis (SCPA) group showed significantly higher i/cPR (1.47+/-0.33) than the bilateral SCPA group (1.07+/-0.21). Comparing the subgroups by the type of IVC inflow, the i/cPR of the intraatrial tunnel group was higher than the hemiazygous continuation group, but there was no statistical significance. CONCLUSION: TCPA does not influence the growth of pulmonary artery, and the type of cavopulmonary anastomosis and the bilaterality of superior vena cava may have major influences on the distribution of the pulmonary blood flow. The details of surgical methods should be evaluated case by case in respect to the associated anomalies in order to achieve adequate postoperative pulmonary blood flow distribution.
Angiography ; Arterial Pressure ; Body Surface Area ; Cardiac Catheterization ; Cardiac Catheters ; Fontan Procedure ; Heart Bypass, Right* ; Hemodynamics ; Humans ; Lung ; Perfusion ; Prognosis ; Pulmonary Artery ; Pulmonary Circulation* ; Vena Cava, Superior

BACKGROUND: Echocardiographic examination is universally considered as an established method for the diagnosis of congenital heart disease, and as a result of many technological advancements in information processing, its utility is being emphasized much more. Cardiac catheterization, by comparison, is usually performed in the past for the purpose of diagnosis and preoperative assessment of infants with isolated ventricular septal defect (VSD), and the risks and complications of cardiac catheterization have been more frequent in the younger ages. Accordingly, we present in this study the effectiveness and safety of echocardiography on the diagnosis and treatment of infants who met the indications of early correction and underwent operations. METHODS: Between May 1994 and April 1997, 66 infants with isolated VSD were submitted for primary correction in the Yonsei Cardiovascular Center. Among the 66 infants (36 males and 30 females), 33 (group 1) underwent surgery on the basis of echocardiography alone and another 33 (group 2), on the basis of cardiac catheterization in addition to echocardiography. The two groups were compared for the diagnostic sensitivity, specificity, complication after surgical correction and frequency of re-operation. RESULTS: 1) The average age was 5.4+/-3.3 months in group 1 and 5.7+/-2.2 months in group 2. 2) There was no significant difference between the two groups in terms of the sensitivity and specificity of diagnostic tools. 3) There was no post-operative death in either group and no significant difference in postoperative hospital stay between the two groups. 4) There was no significant difference between the two groups in complications such as sepsis, pneumonia after surgical correction. CONCLUSION: We concluded that after an accurate selection, most infants with isolated VSD can safely undergo primary repair on the basis of echocardiography alone.
Automatic Data Processing ; Cardiac Catheterization ; Cardiac Catheters ; Diagnosis ; Echocardiography* ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Humans ; Infant ; Length of Stay ; Male ; Pneumonia ; Sensitivity and Specificity ; Sepsis