• Journal of Chest Surgery
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• v.32 no.2
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• pp.108-118
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• 1999

Background: To evaluate the risk factor and long-term result of arterial switch operation , a retrospective study was done. Material and Method: A retrospective analysis was done to evaluate the early and long-term results on 58 patients who underwent an arterial switch operation(ASO) for transposition of the great arteries(TGA) with intact ventricular septum, between January 1988 and December 1996. Beforesurgery, 36 patients(62.1%) underwent balloon atrial septostomy, 32 patients(51.7%) received PGE1 infusion, and preparatory banding of pulmonary artery was performed on 6 patients(mean LV/RV pressure ratio 0.53$\pm$0.11). Result: The age at operation ranged from 1 to 137 days(mean 24$\pm$26 days) and the weights ranged from 1.8 to 6.8 kg (mean 3.5$\pm$0.8 kg). There were 14 early deaths(24.1%), but of the last 24 patients operated on since 1994, there were only 2 early deaths(8.3%). In the risk factor analysis, the date of operation was the only risk factor for early death(p-value < 0.01). Eight of the 14 early deaths were due to acute myocardial failure(mainly inadequate coronary blood flow). The length of follow-up ranged from 2 months to 8 years, average of 36$\pm$27 months. The follow-up included sequential noninvasive evaluations and 21 catheterizations and angiographic studies performed 5 to 32 months postoperatively with particular attention to the great vessel and coronary anastomosis, ventricular function, valvular competence, and cardiac rhythm. There were 5 late deaths(11.4%), one of thesedeaths was related to the late coronary problems, two to aspiration, one to uncontrolled chronic mediastinitis, and one to progressive aortic insufficiency and heart failure. The most frequent postoperative hemodynamic abnormality was supravalvular stenosis and the degree of pulmonary or aortic obstruction had slowly progressed in some cases, however there were no children who had to undergo a reoperation for supravalvular pulmonary or aortic stenosis. Aortic regurgitation was identified in 9 patients, which was mild in 7 and moderate in 2 and had progressed in some cases. Two patients who had an unremarkable perioperative course were identified as having coronary artery obstructions. The other late survivors were in good condition, were in sinus rhythm, and had normal LV functions. Actuarial survival rate at 8 years was 68.8%. Conclusion: We concluded that anatomic correction will be established as the optimal approach to the TGA with intact ventricular septum, though further long-term evaluations are needed.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.18-29
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• 1983

Thirty-two patients with a cyanotic cardiac malformations having more complex intracardiac defects than ones in a tetralogy of Fallot underwent complete intracardiac repairs in a-full-year period from July 1981 to June 1982. Twenty-two patients [68.8%] died within 30 days after surgery: Transposition of the great arteries, seven of 10 patients; Double-outlet right ventricle, four of 6 patients; Tricuspid atresia, four of 6 patients; Single ventricle, all of 4 patients; Pulmonary atresia, two of 3 patients; Double-outlet left ventricle, none of 2 patients; and Truncus arteriosus, one of a single patient. All deaths occurred with a low cardiac output syndrome or a failed off-bypass, and they were almost always accompanied with other grave postoperative complications. The complex intracardiac anatomy itself was one of the risk factors by making a complete intracardiac repair of the defects difficult in a small heart. The reconstruction of the right ventricular outflow carried a difficulty in balancing an adequate relief of the obstruction with an avoidance of making too much pulmonary valvular insufficiency as well. On the other hand, the presence of an elevated pulmonary arterial pressure and a high pulmonary vascular resistance was also the factors affecting the postoperative surviv als. The importance of detailed knowledge of intracardiac anatomy and hemodynamics from the careful preoperative evaluation of the patient was discussed along with the necessity of technical refinement of the correction.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.6
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• pp.594-600
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• 2019

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

• Journal of Chest Surgery
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• v.16 no.4
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• pp.431-443
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• 1983

A hundred and fifty-one patients with congenital heart disease less than 24 months old underwent intracardiac repairs from January 1982 to July 1983, which consists 24.2% of all the patients with congenital heart diseases operated during the same period. There were 98 patients[64.9%] with acyanotic congenital heart disease and 53 patients[35.1%] with cyanotic congenital heart disease, and 55 patients[36.4%] were less than 1 year of age. Twenty-two patients died within 30 days after surgery and 3 patients died after postoperative 30th day: Ventricular septal defect, four of 90 patients; Tetralogy of Fallot, five of 23 patients; Transposition of great arteries, nine of 17 patients; Tricuspid atresia, four of 5 patients; Pulmonary atresia, all of 2 patients; Single ventricle, one of single patients. Over all mortality was 16.6% and mortality of acyanotic congenital heart disease, cyanotic congenital heart disease and patients less then 1 year of age was 4.1%, 39.6% and 20.0% respectively. Still the mortality of cyanotic congenital heart disease is high. Careful preoperative evaluation of the detailed intracardiac anatomy and hemodynamics of the patients and proper selection of surgical treatment yield better clinical results.

• Journal of Chest Surgery
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• v.25 no.4
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• pp.406-411
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• 1992

Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

• 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.37 no.9
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• pp.727-734
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• 2004

The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.759-765
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• 1995

We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.

• Journal of Chest Surgery
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• v.42 no.1
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• pp.87-91
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• 2009

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.263-270
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• 1996

Twenty two patients underwent total anatomic correction of complete atrioventricular septal defect associated with other cardiac anomalies between July 1986 and December 1994. Age ranged from 6 months to 11 years(mean 49.6 $\pm$ 35.8 months), and they were composed of 7 males and 15 females. Combined major cardiac anomalies were tetralogy of Fallot(TOF) in 11 cases, double outlet of right ventricle (DORV) in 6 ca es, and transposition of great arteries (TGA) in 5 cases. Down's syndrome was associated in 5 patients with TOF and 1 patient with DORV. They were classified as Rastelli type A in 3 patients, B in 2 patients, and C in 17 patients. Modified Blalock-Taussig shunt was performed.in 5 patients and Waterston shunt in 1 patient as a palliative procedure. There were 7 perioperative deaths(31.8%) and the causes were pump weaning failure, low cardiac output, acute renal failure, persistant pulmonary hypertension and hypertensive crisis, and sepsis. Reoperations were performed in 4 cases to repair atrioventricular valvular regurgitation or to relieve the right ventricular outflow tract (RVOT) or pulmonary arterial stenosis. One late death was due to aspiration pneumonia. Second reoperation was necessary for progressive worsening of left atrioventricular regurgitation and RVOT stenosis in one patient. Fourteen survived patients were followed up for a mean of 66.0 $\pm$ 26.7months and all of them w re NYHA functional class I or II.