No abstract available.

BACKGROUND: For the evaluation of pulmonary nrtery branches after operation of right ventricular outflow obstructive disease, we reviewed the usefulness of both simple X-ray and lung perfusion scan with 99mTc-macroaggregated albumin compared with cardiac angiography. METHODS: From March, 1986 to July, 1993, at Seoul National University Children;s Hospital, we chose 23 cases who underwent both simple X-ray and cardiac angiography among the patients who had distinct difference between right and left pulmonary blood flow in lung perfusion scan after operation of right ventricular outflow obstructive disease. So we calculated the ratio of right and left pulmonary blood flow and Spearman correlation coefficient. RESULTS: Of the 23 cases, 17 cases were tetralogy of Fallot, 4 cases pulmonary atresia, 1 case double outlet right ventricle, and 1 case truncus arteriosus. The mean age at poeration was 27 months, and the mean postoperative follow-up period was for 68 months. The correlation coefficient between cardiac angiography and simple X-ray was 0.86(p<0.01), and that beteen cardiac angiography and lung perfusion scan was 0.80(p<0.01). CONCLUSION: The cardiac angiography has been known as the best diagnostic tool for the evaluation of pulmonary artery branches after operation of right ventricular outflow obstructive ddisease, but we proposed that the lung perfusion scan should be widely used because its results were similar with those of cardiac angiography.
Angiography ; Double Outlet Right Ventricle ; Follow-Up Studies ; Humans ; Lung* ; Perfusion* ; Pulmonary Artery* ; Pulmonary Atresia ; Seoul ; Tetralogy of Fallot ; Truncus Arteriosus

No abstract available.
Ductus Arteriosus, Patent*

BACKGROUND: In congenital heart disease, the lung perfusion through stenosed pulmonary artery is usually decreased. And this decrement of lung perfusion also occurs with diaphragmatic palsy after the operation of congenital heart disease. It is difficult to delineate the amount of lung perfusion in case of combination of pulmonary artery stenosis and diaphragmatic palsy. We examined the change of lung perfusion after the induction of diaphragmatic palsy in rabbits. METHODS: We dissected left phrenic nerves in 20 rabbits to induce left diaphragmatic palsy. The lung perfusion scan was performed with 99mTc-MAA and the movement of diaphragm was examined with fluoroscopy. They were performed as baseline data and on 3rd and 10th day postoperatively. The amount of left lung pefusion before and after diaphragmatic palsy was compared and analysed in 12 rabbits which definitely had diaphragmatic palsy. RESULTS: Weight of the rabbits was 1.65+/-0.26 kg. Left lung perfusion percent was 45.93+/-6.42% before operation and these were 32.48+/-6.09% and 37.62+/-3.39% on the 3rd and 10th postoperative day, respectively. Left lung perfusion was significantly decreased just after diaphragmatic palsy but it was not changed thereafter. The decrement of lung perfusion was not affected by the body weight. The decreased amount of left lung perfusion was reciprocally correlated with the body weight of the rabbits on the postoperative 3rd day but not 10th day. CONCLUSION: Left lung perfusion percent of the rabbits was decreased 7% with the induction of diaphragmatic palsy and the decreased amount was reciprocally correlated with the body weight just after the diaphragmatic palsy was induced.
Body Weight ; Constriction, Pathologic ; Diaphragm ; Fluoroscopy ; Heart Defects, Congenital ; Lung* ; Paralysis* ; Perfusion* ; Phrenic Nerve ; Pulmonary Artery ; Rabbits

A procedure descried by Fontan and Baudet in 1971 successfully bypassed the right ventricle in Tricuspid Atresia patients, after then many modification of the Fontan operation had been described and applied to many cyanotic complex heart patients. Forty patients with a variety of cardiac malformation underwent the Fontan operation at Seoul National University hospital (September 1978 to June 1986). The age at operation ranged 2 months to 18 years. Each number of cases according to basic cardiac anomaly was as follows; 17 in Tricuspid Atresia, 17 in Univertricular Heart, 2 in Double outlet of Right Ventricle, 2 in Transposition of the Great Arteries and 2 in Criss-cross heart. Total mortality rate after the Fontan operation was 50%. There was only one late death (>30 days). Mortality rate under 4 years of age (67%) was higher than that between 4 and 18 years of age (40%). we observed a significantly higher mortality for patients who, in the immediate postoperative period, had central venous pressure greater than 25cm H2O. 45% among survivals did not require further medication. Although mortality rate after the Fontan operation is much higher than that in the foreign literature, operative mortality will decline with the increased expirence of surgeon and the effective patients selection.
Arteries ; Central Venous Pressure ; Crisscross Heart ; Fontan Procedure* ; Heart ; Heart Ventricles ; Humans ; Mortality ; Postoperative Period ; Seoul ; Tricuspid Atresia

BACKGROUND: Valve replacement in children has many problems such as the durability of prosthetic valve, thomboembolism and hemorrhage. But recently, the necessity of valve replacement in children increased and the above problems were solved party. So the number of valve replacement in children increased progressively. RESULTS: Valve replacement in 47 children were done at Seooul National University Chidren's Hospital from March 1986 to July 1991. The patients were composed of 25 males and 33 females. 25 patients had congenital heart disease and 22 patients rheumatic heart disease. 45 patients received single valve repalcement, 2 patients double valve repalcement, and among all of them, 2 patients redo-replacement. The major valve lesion was mitral insufficiency and post-operative status in view of NYHA functinonal class was improved in most patients. The mechanical valves were applied to 45 patients and tissue valves to 2 patients. The indication of valve replacement were progressive increase in ventricular volume, major regurgitant fraction over grade III decrease in exercise tolerance and vegetation. The overall early mortality was 8.5% and late mortality 0%. There were post-operative complication rate of 30% and late complication rate 14%, and among the later, valve faliure was reported in 2 pantients and thromboembolism in 1 patient. The complication-free rate was 97.7% at post-operative 1 month, 91.3% at 12 months, 90% at 36 months and 60% at 48 months. 43 patients received anticoagulation and/or antiplatelet therapy, but there was no critical indication for this. CONCLUSION: These results suggest that cardiac valve replacement in children have been effective therapeutic modality even though various problems still remain, but we propose that sufficient long-term follow-up and clinical research be needed.
Child* ; Exercise Tolerance ; Female ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Valves* ; Hemorrhage ; Humans ; Male ; Mitral Valve Insufficiency ; Mortality ; Rheumatic Heart Disease ; Thromboembolism

We reviewed 26 cases of cardiac rhabdomyoma in children which were diagnosed between 1986 and 1991 in Seoul National University Children's Hospital. Twenty-two cases were associated with tuberous sclerosis. Total tumor count was 58 and mean tumor count per patient was "daehakgyo".23. Their cardiac rhabdomyomas were diagnosed from fetal period through 12 years after birth. Median age was 9."uiwon" months. Fifteen patients were males and rest 11 patients were females. Cardiac symtoms and signs at initial diagnosis were present in 12 cases. They consisted of cardiac murmur in "hakgyo", arrhythmia in 8 and congestive heart failure in "daehakgyo" cases (one with murmur and the other with arrhythmia). Cardiomegaly was seen in 9 cases on chest X-ray and other "byeongwon" case showed abnormal left cardiac border. Electrocardiography and 24 hour Holter monitorings showed arrhythmia in 15 cases. Clinically significant arrhythmias were found in 7 cases on 24 hour Holter monitorings. They consisted of 1with frequent premature ventricular contractions, "daehakgyo uigwadaehak" with frequent premature atrial contractions, "daehakgyo" with atrial tachycardia and "daehakgyo uigwadaehak" with atrio-ventricular block ("daehakgyo" cases of atrio-ventricular block had other arrhythmias also). The locations of tumors were left ventricle in 21, interventricular septum in 21, right ventricle in 13 and atrium in "daehakgyo uigwadaehak" tumors. Mean tumor count per patient in fetal period and infancy was "daehakgyo".86 and it was statistically much more than that after infancy which was "byeongwon".5. Mean tumor area which was calculated on two dimensional echocardiography was larger in fetal period and infancy(128.9 +/- 38.2mm2)than that after infancy(47.8 +/- 11.2mm2). Mean relative tumor area to aortic root dimension was also significantly larger in fetal period and infancy than that after infancy ("daehakgyo".07vs 0.30). In tuberous sclerosis, mean mass area and mean relartive tumor area to aortic root dimension in fetal period and infancy was significantly larger than those in patients after infancy. Of 26 cases, tumorectomy was done in "daehakgyo uigwadaehak" cases due to hemodynamic obstruction and arrhythmia. One case died immediately after operation and the other "daehakgyo" cases are still alive with the follow up period of 30 and 44 months each. Of the 7 patients with significant arrhythmia on 24 hour Holter monitorings, pacemaker insertion was done in "byeongwon" case who received tumorectomy and other 1 case was treated with anti-arrhythmic drugs. All the other patients did not need anti-arrhythmic drugs or other management.
Arrhythmias, Cardiac ; Atrial Premature Complexes ; Cardiomegaly ; Child* ; Diagnosis ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Failure ; Heart Murmurs ; Heart Ventricles ; Hemodynamics ; Humans ; Male ; Parturition ; Rhabdomyoma* ; Seoul ; Tachycardia ; Thorax ; Tuberous Sclerosis ; Ventricular Premature Complexes

No abstract available.
Child* ; Heart* ; Humans

OBJECTIVE: The purpose of this study was to evaluate the therapeutic efficacy and toxicity of high dose cisplatin-cyclophosphamide combination chemotherapy on patients with primary epithelial ovarian cancer. METHODS: A review of 63 patients previously diagnosed as primary epithelial ovarian cancer after initial operation and histology at Pusan National University Hospital from Jul. 1993 to Jun, 1997 was performed. Patients were received the combination chemotherapy including cisplatin 100mg/m2/day and cyclophosphamide 750mg/m2/day, repeated 6 cycles every 4 weeks. The mean age was 48 years old, and previous surgical procedures were total abdominal hysterectomy and bilateral salpingo-oophorectomy with omentectomy. The patients were classified into FIGO stage and pathologic results. RESULTS: The clinical response rate was 100% in the FIGO stage Ic patients with PC combination chemotherapy, 100% in stage II, 53.5% in stage III, and 25% in stage IV. The overall response rate was 69.8%. The 3-year survival rate according to the treatment groups was 93.3% in stage Ic group, 60% in stage II, 50% in stage III and 0% in stage IV. The mean survival duration was 34.6 months. Hematologic toxicities in cisplatin-cyclophosphamide chemotherapy were neutropenia and anemia. Nausea and vomiting were the most common side effects and occurred in 96.8%. Most of the toxicities were grade 1 and 2. CONCLUSION: The combination chemotherapy with cisplatin-cyclophosphamide is relatively safe and effective method in the treatment of primary epithelial ovarian cancer.
Anemia ; Busan ; Cisplatin* ; Cyclophosphamide* ; Drug Therapy ; Drug Therapy, Combination* ; Humans ; Hysterectomy ; Middle Aged ; Nausea ; Neutropenia ; Ovarian Neoplasms* ; Survival Rate ; Vomiting

BACKGROUND: The development and progression of a tumor can be determined by a complex multistep process involving the activation of oncogenes and the inactivation of tumor suppressor genes. The purpose of this study is to investigate the expression of p53, Rb, bcl-2 protein and Ki-67 labeling index in the intrahepatic cholangiocarcinoma. METHODS: We analyzed 36 cases of intrahepatic cholangiocarcinoma obtained by surgical resection. Expression of p53, Rb, bcl-2 proteins and Ki-67 labeling index were evaluated by immunohistochemical study. RESULTS: Expression of p53 protein was detected in 61.1% (22/36) of cholangiocarcinoma. Rb protein loss and overexpression were observed 27.8% (7/36) and 72.2% (29/36) of cholangiocarcinoma. But bcl-2 protein was not expressed. No significant correlation was found between p53, Rb and bcl-2 protein expression and age, sex, gross type, histologic grade, vascular invasion and lymph node metastases. The Ki-67 labeling index was significantly higher in p53 positive group and Rb overexpression group than in p53 negative group (p<0.01) and Rb loss group (p<0.05). There was a positive correlation between p53 protein and Rb protein expressions, but a negative correlation between Rb protein and bcl-2 protein expressions. CONCLUSIONS: The overexpression of p53 protein and Rb protein may be closely associated with cholangiocarcinogenesis, while bcl-2 has a less crucial role in cholangiocarcinogenesis.
Cholangiocarcinoma* ; Genes, Tumor Suppressor ; Lymph Nodes ; Neoplasm Metastasis ; Oncogenes ; Retinoblastoma Protein