• Journal of Chest Surgery
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• 제56권4호
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• pp.290-293
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• 2023

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart disease that often needs a critical decision on whether to open the right ventricular outflow tract (RVOT). Significant morbidity and considerable mortality might preclude the safe use of percutaneous or surgical right ventricular decompression in patients with muscular PAIVS. We report the case of a 21-day-old neonate weighing less than 3 kg who underwent hybrid RVOT stent insertion as initial palliation for muscular PAIVS and subsequent anatomical correction at 5 months of age, with 6 years of follow-up.

• Journal of Chest Surgery
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• 제6권1호
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• pp.63-68
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• 1973

Two patients operated upon for tricuspid atresia by Glenn operation are presented. They were five years old and four years old females who were cyanotic shortly after birth, and remained cyanotic. On physical examination, cyanosis on digits and lips, clubbing of fingers, thrill and grade III systolic murmur on 4th I.C.S. along left sternal border were noted. In the first case, chest roentgenograms showed normal pulmonary markings, and the electrocardiogram was interpreted as showing left ventricular hypertrophy with left axis deviation and peaked P-wave in lead IL Right heart catheterization showed high pressure in right atrium and the catheter tip was easily inserted into the left atrium through septal defect. In the second case, chest roentgenograms showed cardiomegaly with sparse pulmonary vascular markings and narrowed vascular pedicle, and the electrocardiogram showed left ventricular hypertrophy with some element of right atrial hypertrophy. Angiocardiogram showed changes characteristic of tricuspid atresia, including "right ventricular window" The findings of right heart catheterization were similar to those of first case. On the basis of these observations, they were diagnosed as tricuspid atresia, and Glenn operation was performed. Normal position of great vessels combined with ventricular septal defect and pulmonary stenosis were noted on the first case, and on second case, transposition of great vessels was additional finding. Postoperative course was uneventful and favorable outcome was obtained.

• Journal of Chest Surgery
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• 제24권11호
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• pp.1149-1153
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• 1991

A modified procedure for the operative management of the corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis or atresia was performed in 4 patients. (age ; 18 months-9 years). The operation consists of a venous switch procedure (Senning), intraventricular baffling directing the blood flow from the morphologic left ventricle into the aorta via ventricular septal defect through the right ventriculotomy, and insertion of valved conduit between the morphologic right ventricle and the pulmonary artery. All the four patients had large non-restrictive ventriular septal defects and no evidence of significant mitral valve regurgitation. Both ventricles were well-developed. Three cases had pulmonary atresia, and the remainder had severe subpulmonic stenosis. Postperatively, all patients showed no findings of systemic or pulmonary venous obstruction withnormal sinus rhythm. One patient died of sepsis due to infection by Methicillin-resistant S. aureus on the thirteenth postoperative day. Follow-up is done on the survivors, and all of them are dong well with regular sinus rhythm, with their functional class I or II at 2 to 8 months after the operation.

• Journal of Chest Surgery
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• 제23권5호
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• pp.953-961
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• 1990

Congenitally corrected transposition of the great arteries is a rare congenital heart anomaly, in isolation, has no hemodynamic consequences. It is usually associated with one or more of a variety of intracardiac lesions, ventricular septal defect, valvular or subvalvular pulmonary stenosis, and deformity of the systemic atrioventricular valve with insufficiency. This report describes a successful two stage operation for congenitally corrected transposition, [SLL] type, with ventricular septal defect, pulmonary atresia, persistent ductus arteriosus, and atrial septal defect. A 9 years old patient underwent modified Blalock-Taussig operation because of severe pulmonary hypoplasia. 2 years later a corrective operation, direct closure of ASD and PDA, VSD closure with Dacron patch, Enlargement of left pulmonary artery with pericardial patch and Relief of ROTO with Rastelli procedure could be successfully performed without complication.

• Journal of Chest Surgery
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• 제33권3호
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• pp.257-261
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• 2000

Pulmonary arteriovenous fistula can occur in a variety of clinical situations including liver diseases, infections, metastatic carcinomas, systemic disorders, and after the palliation of congenital heart diseases. A 72-day-old male infant with Tetralogy of Fallot and pulmonary atresia underwent surgical correction without difficulty. However, ventilator weaning in the ICU failed initially because of an unexplained postoperative hypoxemia(FiO2: 0.8, PaO2: 40 mmHg, SaO2: 80∼90%). Postoperative follow-up lung perfusin scan at postoperative 15 days showed right-to-left shunt(33.6%) and ventilator weaning was performed on the 20th day after the operation (FiO2: 0.4, PaO2, 50mmHg, SaO2: 86.9%). Arterial oxygen saturation under room air was 80∼85% at 7 months postoperatively. One and half year follow-up lung perfusion scan showed decreased amount of right-to-left shunt (11.2%). We report a case with a review of the literatures.

• Journal of Chest Surgery
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• 제18권4호
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• pp.569-573
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• 1985

A 18-year-old female underwent surgical correction of tricuspid atresia and complete transposition of great arteries combined with atrial 8 ventricular septal defect and pulmonary stenosis. After the transection of main pulmonary artery just above the pulmonic valve, proximal portion of main pulmonary artery was closed with running suture and distal portion of main pulmonary artery anastomosed with right atrial appendage without valve insertion. Atrial septal defect was closed with running suture. Postoperative course was uneventful and she discharged on 18th postoperative day. Her condition is in very good until present. Modified Fontan`s operation without valve placement [in the condition of low pulmonary vascular resistance and good left ventricular function] may has a good result.

• Journal of Chest Surgery
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• 제23권6호
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• pp.1191-1203
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• 1990

Pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries [abbreviated as PA+VSD+MAPCA in the following] has limited the success of attempts at accurate diagnosis and complete surgical repair. From April 1986 to September 1990, 23 patients with PA+VSD+MAPCA among 96 patients of PA+VSD in Seoul National University Children’s Hospital were encountered. The group comprised 14 male and 9 female patients with ages ranging from 17 days to 177 months [mean 49.6 months]. We operated one stage total repair on good pulmonary artery sized two patients by R.E.V. [Reparation a l’etage ventriculaire] and Rastelli operation respectively. And the 11 patients who had independent MAPCAs and hypoplastic central pulmonary artery were dealt with unifocalization and modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients later. We successfully had managed 7 patients whose MAPCAs could be ligated with modified Blalock-Taussig Shunt and followed by second stage repair in 3 patients with R.E.V or Rastelli operation. Recently, three obstruction after 11 unifocalization procedures made us to try palliative right ventricle-pulmonary artery conduit operation by Gore-Tex vascular graft interposition under cardiopulmonary bypass. And so we managed another 3 patients with these procedures for the purpose of pulmonary artery growth whose central pulmonary artery were severely hypoplastic. We experienced one death after second stage repair whose central pulmonary artery was created by 12mm Gore-Tex vascular graft and was unifocalized.

• Journal of Chest Surgery
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• 제19권2호
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• pp.306-312
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• 1986

The development of extracardiac valved conduits for establishment of RV-PA continuity has permitted repair of previously uncorrectable congenital heart anomalies and has facilitated the repair of other complex lesions. But the distressing problem of neointimal peel formation with eventual conduit obstruction in patients with Dacron valved conduits has led to the need for premature replacement in many patients. Therefore in the absence of pulmonary hypertension, hypoplastic pulmonary arteries, significant right ventricular dysfunction or unrepaired tricuspid regurgitation, preference of a valveless conduit to a valved conduit gives excellent results and may diminish the need for late reoperation. In our Hanyang University Hospital, the Rastelli operation was performed for the repair of pulmonary atresia with a valveless Gore-Tex conduit. The patient was operated on with good result. The CVP after operation was 8-13 mmHg at POD #0 and 4-6 mmHg from POD #3-4. Postoperatively the patient was acyanotic and had improved physical capacity compared with his preoperative status.

• Neonatal Medicine
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• 제18권2호
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• pp.365-369
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• 2011

폐동맥폐쇄가 동반된 팔로네증은 팔로네증 중에 가장 심한 형태이다. 이런 종류의 선천성 심질환에서 폐혈액 순환을 지속하기 위하여 동맥관 개존을 유지하는 것은 생명을 구하는 매우 중요한 치료이다. Milrinone은 선택적 인산이에스테르효소 3 억제제이며 강력한 혈관 확장제로 알려져 있다. 저자들은 폐동맥 폐쇄가 동반된 팔로네증 신생아에서 폐순환을 지속시키기 위하여 동맥관 개방을 유지하는 치료로 milrinone을 성공적으로 사용한 증례를 보고한다. Milrinone은 동맥관 개방효과뿐 아니라, 심근수축 촉진, 이완 촉진, 폐혈관 확장 효과가 있으며 부작용은 심하지 않아 더 이로운 치료방법이 될 가능성이 있다. Milrinone의 치료효과를 밝히기 위하여 더 많은 치료증례 조사와 타 약제와의 비교 연구 등이 필요하다.

• Journal of Chest Surgery
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• 제23권5호
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• pp.936-943
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• 1990

The Fontan operation for tricuspid atresia was first performed in 1968[Fontan and Baudet 1971] and several technical modifications of procedure were developed. We have experienced a case of modified Fontan operation for tricuspid atresia type Ic who was 5 years old female. She had previous palliative procedure \ulcornerpulmonary artery banding due to excessively increased pulmonary blood flow. The modified Fontan operation was right atrium to right ventricle connection with valve-bearing conduit. The result was good and the patient was discharged 20 days after operation.