• Journal of Chest Surgery
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• v.34 no.11
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• pp.854-857
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• 2001

Double-outlet left ventricle(DOLV) is a rare congenital cardiac malformation, defined as the origin of both the aorta and the pulmonary artery being entirely or predominantly above the morphologically left ventricle, which is difficult to diagnose accurately. A 3-year old male was admitted for cyanosis and dyspnea. At the age of 2 months, he had undergone pulmonary artery banding and coarctoplasty. He was diagnosed as DOLV with subaortic ventricular septal defect(VSD). Biventricular repair was achieved by patch closure of VSD, primary closure of PFO, and pulmonary trunk translocation from left ventricle to right ventricle. The advantages of this procedure using native tissue for right ventricular outflow tract reconstruction are growth potential and preserved valve function, which contribute to a decreased likelihood of reoperation related to the right ventricular dysfunction related to pulmonary insufficiency.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.145-149
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• 2006

The surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis remains a challenge. The Rastelli operation or Lecompte operation is the preferred surgical procedure, but its long-term results are not optimal because of a warped left ventricular outflow tract through a space-occupied intraventricular tunnel and a contrived right ventricular outflow tract. We performed a half-turned truncal switch operation as an alternative surgical procedure in a 3-year-old boy (weighing 9.6 kg) with this anomaly. Postoperative echocardiography showed laminar flow through straight and nonobstructive aortic and pulmonary ventricular outflow tracts.

• Journal of Chest Surgery
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• v.43 no.1
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• pp.58-62
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• 2010

Rastelli repair has been considered the procedure of choice for surgically repairing transposition of the great arteries combined with ventricular septal defect and pulmonary stenosis. However, the long term results have been less than optimal and these patients who ungo this procedure can eventually display conduit obstruction, left ventricular outflow tract obstruction and arrhythmias. Many new procedures are now available and they are technically challenging for making a more normal anatomic repair. In our hospital, two patients who had a TGA with VSD and PS have been repaired with a Half Turned Truncal Switch Operation and a Lecompte maneuver in 2003 and 2006, respectively. We report on our two experiences with performing a Half Turned Truncal Switch Operation, and we discuss the changes of the biventricular outflow tract.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.417-422
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• 2008

Background: The aim of this study is to evaluate the long term results of creating various right ventricle to pulmonary artery conduits for treating complex congenital heart disease. Material and Method: Between June 1986 and July 2006, we retrospectively reviewed 245 patients who underwent reconstruction of the right ventricular outflow tract with various kinds of conduits. 410 operations were done in 245 patients, the mean age at operation was $3.2{\pm}4.9$ years (range: 7 days$\sim$45 years) and the mean body weight was $12.5{\pm}8.7\;kg$ (range: $2.4\sim76.3\;kg$). Result: We used the following conduits: Polystan conduit, Shelhigh conduit, Carpenter-Edward conduit, Dacron graft with an artificial valve, valveless Gore Tex vascular graft, homograft and hand-made bovine or autologous pericardial conduit. The mean follow up duration was $6.3{\pm}5.2$ years. Redo operation for RV-PA conduit dysfunction was performed in 131 patients, a second redo was done in 31 and a third redo was done in 3. The reoperation free rates were 67.3%, 48.5% and 39.4% for 5 years, 10 years and 15 years, respectively. The homograft showed the best durability, followed by the Dacron graft with artificial valve and the Carpentier-Edward conduit. The larger sized conduit showed better durability. Conclusion: The homograft showed lowest reoperation rate and a smaller size of conduit showed the highest reoperation rate. The reoperation rate for the RV-PA conduit was about 35% at 5 years, so it is mandatory to develop the more durable conduit for RV outflow.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.189-193
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• 2003

The patient was a 30-day-old female infant with symptoms of severe dyspnea and cyanosis, when she was admitted to the ER. The echocardiography revealed DORV with subpulmonary VSD, and the diagnosis of Taussig-Bing anomaly was made. Two days after admission, an urgent operation was performed. The operation consisted of intraventricular tunnel repair and arterial switch operation. She was discharged, and after checking her chest X-ray through OPD, there was no interval change of cardiomegaly. She was then re-admitted, and the angiography revealed coactation of aorta. We performed a resection and end-to-end anastomosis of aorta. She is currently in good condition 11 months postoperatively.

• Journal of Chest Surgery
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• v.32 no.7
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• pp.621-627
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• 1999

Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.660-667
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• 1998

Background: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months(range, 3 to 83). Of these patients, 28(63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis(or pulmonary atresia), 14(31.8%) had double outlet right ventricle with pulmonary stenosis(or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. Results: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions(4 cases), residual muscular ventricular septal defect(1 case), and recurrent septic vegetation(1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance(p<0.05) and all the risk factors for the recurrent pulmonary stenosis such as age, pulmonary artery index, and materials used for the pulmonary outflow tract reconstruction had no statistical significance(p>0.05). Conclusions: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.154-161
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• 2003

Purpose : The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. Methods : Medical records of 94 patients(males 58, females 36; mean age at last follow-up, $12{\pm}9$ years; mean follow-up duration, $9{\pm}6.4$ years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. Results : Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P=0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. Conclusion : TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

• Journal of Chest Surgery
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• v.36 no.6
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• pp.384-390
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• 2003

Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.

• Journal of Chest Surgery
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• v.34 no.10
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• pp.754-762
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• 2001

Background: To evaluate the efficacy of arterial switch operation for transposition of great arteries, serial echocardiographic studies were performed in 8 patients who underwent the surgery between 1989 and 1998 at Dept. of Thoracic & Cardiovascular Surgery, Yeungnam University Hospital in Daegu City, Korea. Material and Method: Follow-up period ranged from 6 months to 11 years(average of 4.1 years). Body weight ranged from 2.6kg to 4.8kg, with average of 3.6kg. 5 of 8 patients were preoperatively diagnosed as TGA+VSD, and 3 as TGA+IVS. LV function was evaluated by the measurement of LV shortening fraction, LVSTI, and LVEF. RVSTI was also measured. Postoperative function of valve and growth of great vessels were analyzed by the measurement of PSPGV, valvular regurgitation, LA/AO ratio, root dimension of aorta and pulmonary artery, comparing with the age matched controls, respectively. Result: LVEF had an average of 65.0+9.03% which is tended to increase serially. LVAOPG had an average of 15.9mmHg. RVPAPG, 27.5mmHg. From the measurement of aortic root dimension of 6 patients at end-systole, aortic root growth was assumed to increase more than the mean value of normal growth. PA root dimension at end-systole showed a similar growth progress when compared with age matched normal controls. Postoperative pulmonic valve regurgitation was noted in 5 of total 8 patients, in which 1 patient who showed grade 2 and 4 showed below grade 1. AR, in 6 patients and all grade 1 Except 1 patient, all the valvular regurgitations were below grade 1, which was presumed to be clinically insignificant.