• Journal of Chest Surgery
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• v.31 no.9
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• pp.861-866
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• 1998

Background: Pulmonary atresia (PA) with ventricular septal defect has various morphology of pulmonary arteries and pulmonary blood flow sources, so pulmonary arterial hypoplasia and arborization abnormality make this anomaly difficult to manage surgically. In cases associated with juxtaductal stenosis, we evaluated the change of the pulmonary arterial and juxtaductal stenotic site after shunt operations, and would like to find useful information in surgical planning and methodology of these patients. Material and Method: Among 59 cases diagnosed as PA with ventricular septal defect associated with juxtaductal stenosis, 29 cases who had cardiac catheterization before and after shunt operation were selected from July, 1991 to July, 1996. In 10 cases of right shunt operation(Group I) and 19 cases of left shunt operation (Group II), the diameters of the descending aorta, both pulmonary arteries, and the juxtaductal stenosis site were measured before and after the shunt operation. Result: In both Group I and II, the pre- and postoperative ratio of diameters of the ipsilateral pulmonary artery to the descending aorta was from 0.78${\pm}$0.31 units to 1.01${\pm}$0.26 units and from 0.67${\pm}$0.18 units to 0.84${\pm}$0.27 units respectively, showing a signigicant increase. The contralateral pulmonary artery index was increased from 0.92${\pm}$0.28 units to 1.05${\pm}$0.15 units and from 0.94${\pm}$0.27 units to 1.08${\pm}$0.37 units respectively, but could not be confirmed statistically. In both groups, the change of juxtaductal stenosis showed an aggravating tendency but of no statistical significance from 0.43${\pm}$0.27 units to 0.39${\pm}$0.25 units and from 0.32${\pm}$0.10 units to 0.30${\pm}$0.16 units respectively, and we experienced 2 total obstruction in Group II. Because the increased pulmonary blood flow by shunt operation has a favorable effect to the pulmonary arterial growth, the shunt operation is a recommended treatment in patients with hypoplastic pulmonary arteries. But in PA with ventricular septal defects, the change of juxtaductal stenosis is very important. In conclusion, the growth of ipsilateral (shunt site) pulmonary artery was promoted by shunt operation, but there is a tendency for the juxtaductal stenosis to be aggravated. And we experienced 2 total obstruction in Group II. Conclusion: Thus, in cases operated with shunt method, much careful postoperative follow up study including angiographic evaluation is needed, and after the shunt operation on the side of pulmonary artery associated with juxtaductal stenosis, early precise planning for total correction is recommended.

• Proceedings of the KTCVS Conference
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• 1995.10a
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• pp.7-7
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• 1995

• Journal of the Korean Society of Radiology
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• v.83 no.2
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• pp.372-377
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• 2022

Unilateral pulmonary vein atresia (PVA) is a rare congenital cardiovascular anomaly occurring after the common pulmonary vein fails to incorporate into the left atrium. It is most commonly diagnosed in childhood, and diagnosis after reaching adulthood is extremely rare. Dyspnea on exertion and hemoptysis are common clinical features in adult PVA patients, whereas lung parenchymal abnormalities are indirect signs of PVA, which can manifest as interstitial lung disease. Herein, we present the case of a 62-year-old female diagnosed with unilateral PVA presenting as unilateral interstitial lung disease and report the changes in her chest radiographs over 12 years.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.314-319
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• 1985

Nineteen patients with various types of cyanotic congenital heart disease underwent systemic-pulmonary artery shunts with a microporous polytetrafluoroethylene [PTFE] graft between September, 1983, and April, 1985. Age ranged from 3 months to 18 years, and seven of them were less than 12 months old. There were seventeen Great Ormond Street type of modified Blalock-Taussig shunts, and two central polytetrafluoroethylene shunt [ascending aorta-right pulmonary artery]. There was one postoperative death [1/19=5.3%] in a 10 Kg child born with pulmonary atresia and ventricular septal defect associated with patent ductus arteriosus. He had another anomaly of imperforated anus. Relief from cyanosis was achieved in other eighteen patients with variable degree. Eighteen survivors have been followed up from 1 month to 19 months. Clinical status, auscultation, oxygen partial pressure of arterial blood, and hemoglobin have been used to establish shunt patency in all survivors. By above criteria, all survivors have good patent shunt.

• The Korean Journal of Nuclear Medicine
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• v.34 no.2
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• pp.154-158
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• 2000

This is a case report of a 5-month-old male who was brought in to hospital for evaluation of jaundice from birth. The baby had a history of ileal atresia operated 2 days after birth. At the age of one month, Tc-99m DISIDA hepatobiliary scintigraphy was performed at other hospital and reported to show good hepatic uptake of the tracer but no uptake in the biliary tree, gall bladder, or intestine for 24 hours post injection. He was judged to have biliary atresia. However, subsequent exploratory laparotomy revealed that the hepatobiliary tree appeared intact and that there was a gall bladder. Additionally, the patient had central aorto-pulmonary shunt for the right ventricular septal defect with pulmonary stenosis of a peripheral type at the age of 4 months. The second hepatobiliary scintigraphy was performed on admission at the age of 5 months, showing a gall bladder but no intestinal uptake up to 24 hours. Retrospectively, the histological specimen of the liver obtained at the exploratory laparotomy was re-evaluated, and by the histological findings coupled with clinical data, arteriohepatic dysplasia (Alagille's syndrome) was diagnosed. In this report, we emphasize the diagnostic limitation of hepatobiliary scintigraphy and the importance of overall clinical and histologic evaluation in a case of Alagille's syndrome.

• Journal of Chest Surgery
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• v.23 no.2
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• pp.213-221
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• 1990

We have experienced 62 cases of modified Fontan operations in Seoul Nat’l University Hospital from Apr. 1986 to Jul. 1989, They were 38 males and 24 females, and their age was ranged from 16 months to 15.5 years of age. [mean age : 5.73$\pm$2.99 years] There were 16 operative deaths and 2 late deaths, therefore 29% of overall mortality. Their diagnoses were as follows; 28 single ventricle, 11 tricuspid atresia, 6 DORV with LV hypoplasia, 3 pulmonary atresia with hypoplastic RV, 3 TGA with hypoplastic RV, 3 cor\ulcornerGA with hypoplastic LV and PA, 6 AV canal defects with PA, and 2 others. Low cardiac output and pleural effusion were developed frequently, so we divide 40 patients into some groups to analyze the physiologic and anatomic causes of them. By the degree of the LCO, group A was no LCO[mean amount of inotropics used: 0-5 \ulcornerg/kg/min] with 17 cases, B mild LCO [5-10] with 11, C moderate to severe LCO but alive[>10] with 8, D severe LCO to death with 4 cases. For the pleural effusion, group 1 was to be removed the chest tube within 1 week with 8 cases, group II within 3 weeks with 21 cases, group III beyond 3 weeks with 12 cases. We considered their age, diagnosis, pulmonary artery size[PA index], pulmonary artery abnormality, palliative shunt, systemic ventricular type, pulmonary artery wedge pressure, as preoperative factors, and operative methods, and as postoperative factors, CVP, LAP, arrhythmia, thrombosis, atrioventricular valvular insufficiency, etc. In the view of LCO, pulmonary artery size and PCWP were statistically significant [P<0.05], and arrhythmia, A-V valve insufficiency were inclined to the group C and D Pleural effusion was influenced by the pulmonary artery size, pulmonary artery resistance, PCWP, and CVP significantly. [P<0.05] And arrhythmia, residual shunt, and A-V valvular insufficiency were inclined to group II and III, too. As a results, the followings are to be reminded as the important factors at the care of post-Fontan LCO, and persistent pleural effusion [1] pulmonary artery size, [2] pulmonary artery resistance, [3] PCWP, [4] CVP, [5] arrhythmia, [6] residual shunt, [7]A-V valvular insufficiency.

• Advances in pediatric surgery
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• v.14 no.1
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• pp.1-11
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• 2008

The aim of this study is to analyze the outcomes of the esophageal atresia with tracheoesophageal fistula over the last 2 decades. The records of 51 patients born between 1987 and 2006 were reviewed. Twenty-seven patients were male. Mean values of the age, gestational age and birth weight were 2.9 days, 296 days and 2.7kg, respectively. All patients had Gross type C anomalies. Thirty-one patients (60.7 %) had one or more associated congenital anomalies and the most common anomaly was cardiac malformation. In 48 cases, primary anastomosis was done and staged operation was done in one case. Circular myotomies in the proximal esophagus were performed in 9 cases. Postoperative complication developed in 26 cases (54 %): pulmonary complication in 12 cases, anastomotic leakage in 10 and anastomotic stricture in 10, recurrent trachoesophageal fistula in one and tracheomalasia in 2 cases. Reoperation was carried out in 2 patients with anastomotic leaks, the remaining leaks were managed non operatively. Three of the strictures were reoperated upon and the others were successfully managed by balloon dilatations. Overall mortality rate was 15.6 %. Mortality rate of the second 10 years (8 %) period decreased significantly compared to that of the first 10 years (23 %) period.

• Journal of Chest Surgery
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• v.32 no.12
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• pp.1135-1139
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• 1999

Multistage unifocalization and complete repair have been performed for pulmonary atresia ventricular septal defect and major aortopulmonary collateral arteries. We reported a case that divided major aortopulmonary collateral artery was changed into an aneurysm that compressed the left main bronchus. A 1-year-8-month old boy was operated. The Rastelli operation with left pulmonary artery reconstructuion ligation of patent ductus arteriosus and take-down of right Blalock-Taussing shunt was performed on the patient who had pulmonary atreisia ventricular septal defect patent ductus arteriosus and MAPCA at 1 year and 8 months of his age. He previously underwent the unifocalization and right B-T shunt at 9 months of age,. He repeatedly had difficulty in weaning from the mechanical ventilator, After removing the aneurysm from the divided MAPCA that compressed the left main bronchus externally it was possible to wean him from the mechanical ventilator.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.518-521
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• 2010

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.1 no.1
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• pp.115-119
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• 1998

Congenital bronchoesophageal fistula associated esophageal atresia usually presents in the newborn period or infancy but those without esophageal atresia are more insidious in disease process. Symptoms which include cough, hemoptysis, choking on swallowing liquids, uncommonly dysphagia, and epigastric discomfort may not begin until adult life. Most of the cases are curative unless there are serious underlying conditions. The diagnosis is usually made by gastroesophagoscopy, esophagogram, bronchogram and bronchoscopy. And the most of the cases can be cured by fistulectomy and resection of involved pulmonary lobes. We experienced one case of congenital bronchoesophageal fistula which occurred in a 13- year-old girl who complained of paroxysmal cough and intermittent hematemesis for 3 years.