• Journal of Chest Surgery
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• 제31권9호
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• pp.861-866
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• 1998

심실중격결손이 동반된 폐동맥폐쇄(PA/VSD)는 폐동맥의 형태 및 공급원이 매우 다양하고, 폐동맥의 발육부전 과 협착 및 폐동맥지 연결이상 유무가 교정수술시의 문제점으로 대두되고 있으므로, 저자들은 동맥관인접협 착(juxtaductal stenosis)이 동반되어 있는 환자들을 대상으로 체폐단락술전후 폐동맥 크기의 변화를 비교하 여, 수술방법 및 시기를 결정하는데 도움이 되고자 본 연구를 하였다. 1991년 7월부터 1996년 7월까지 연세대 학교 심장혈관센터에서 심실중격결손이 있는 폐동맥폐쇄환자중 동맥관인접협착이 동반되어 체폐단락술을 시행 한 59례가 있었으며, 수술전후의 심도자술 및 심혈관조영술을 시행한 29례를 대상으로 하였다. 우측(10례, Group I) 및 좌측단락술(19례, Group II)에서 단락술전후의 하행대동맥, 양측폐동맥 및 동맥관인접협착 부위의 직경을 측정하여, 수술부위에 따른 수술전후 변화를 비교하였다. 두 군에서, 하행대동맥직경에 대한 동측 폐동 맥직경의 비(ratio)가 수술전 0.78$\pm$0.31에서 수술후 1.01$\pm$0.26로, 또한 수술전 0.67$\pm$0.18에서 수술후 0.84$\pm$ 0.27로 각각 유의하게 증가하였으며, 편측 폐동맥직경의 비는 수술전 0.92$\pm$0.28에서 수술후 1.05$\pm$0.15로, 또한 수술전 0.94$\pm$0.27에서 수술후 1.08$\pm$0.37로 각각 증가하였으나 통계학적으로 유의하지는 않았다. 동맥관 인접협착 부위의 변화는 수술전 0.43$\pm$0.27에서 수술후 0.39$\pm$0.25로, 또한 수술전 0.32$\pm$0.10에서 수술후 0.30$\pm$0.16로 감소하는 경향을 보였으나 통계학적으로 유의하지 않았으며, 2례의 경우에서는 수술후 단절된 소견을 보였다. 체폐단락술을 통한 폐동맥혈류의 확보는 폐혈관 성장에 좋은 효과가 있으므로 폐혈류 감소 및 폐동맥 발육부전환자에서 추천되는 치료법이나 심실중격결손을 동반한 폐동맥폐쇄의 경우에는 동맥관인접협착의 변화가 중요한 것으로 생각된다. 본 연구 결과에서는 체폐단락술을 시행한 동측의 폐동맥이 성장함을 확인할 수 있었고, 동맥관인접협착은 악화될 가능성이 있는 것으로 생각되었다. 또한, 좌측 체폐단락술을 시행하여, 2례의 좌측 폐동맥의 단절을 경험하였다. 그러므로, 체폐단락술을 시행한 경우에는, 보다 철저한 술후 추적 및 검사가 요구되며, 동맥관인접협착이 동반된 측의 폐동맥에 단락술을 시행한 경우에는 조기에 완전 교정술을 고려하여야 할 것으로 사료된다.

• 대한흉부심장혈관외과학회:학술대회논문집
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• 대한흉부외과학회 1995년도 제27차년차학술대회 및 총회
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• pp.7-7
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• 1995

• 대한영상의학회지
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• 제83권2호
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• pp.372-377
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• 2022

일측성 폐정맥 폐쇄는 총폐정맥이 좌심방내로 연결되는 못하는 드문 심혈관계 기형이다. 일측성 폐정맥 폐쇄는 흔히 어린 시기에 진단이 되며, 성인이 된 이후에 진단이 되는 경우는 극히 드물다. 성인 환자에서 활동 시 호흡곤란과 객혈이 흔한 임상증상이다. 폐실질의 이상은 폐정맥 폐쇄의 간접적인 소견이며, 간질성 폐질환으로 나타날 수 있다. 우리는 62세 여자 환자에서 일측성 간질성 폐질환을 보이는 일측성 폐정맥 폐쇄의 증례와 12년간의 흉부 X선 사진 소견의 변화를 보고하고자 한다.

• Journal of Chest Surgery
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• 제18권2호
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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.

• 대한핵의학회지
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• 제34권2호
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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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• 제23권2호
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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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• 제14권1호
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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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• 제32권12호
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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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• 제43권5호
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• pp.518-521
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• 2010

생후 1일된 환아가 청색증과 심잡음을 주소로 전원되었다. 심장 초음파 검사를 시행한 결과, 폐동맥 폐쇄가 동반되어 동맥관 의존성 폐혈류를 보이는 증상이 심한 엡스타인 기형(Carpentier type C)이었다. 심방화된 우심실의 벽은 매우 얇고 수축력이 저하되어 있었으며 기능적 우심실의 크기가 매우 작고 폐동맥 폐쇄가 동반되어 양심실 교정이 불가능하다고 판단하였다. 생후 1개월 째 우심방 절제 성형술, 심방화된 우심실의 광범위한 절제 후 봉합 폐색, 변형 Blalock-Taussig 단락술을 시행하였다. 환아는 별다른 문제 없이 퇴원하였으며 생후 5개월에 양방향성 상대정맥-폐동맥 단락술을 시행받았다. 저자 등은 심한 증상을 나타내는 신생아 엡스타인 기형에서 우심실 제외 수술을 시행하여 좋은 결과를 얻었기에 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제1권1호
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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.