• Journal of Chest Surgery
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• v.57 no.3
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• pp.312-314
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• 2024

A 3-year-old boy with Glenn physiology exhibited refractory heart failure with reduced ejection fraction. To improve the patient's oxygen saturation, he underwent ventricular assist device (VAD) implantation with concomitant Fontan completion. The extracardiac conduit Fontan operation was performed with a 4-mm fenestration. For VAD implantation, Berlin Heart cannulas were positioned at the left ventricular apex and the neo-aorta. Following weaning from cardiopulmonary bypass, a temporary continuous-flow VAD, equipped with an oxygenator, was utilized for support. After a stabilization period of 1 week, the continuous-flow VAD was replaced with a durable pulsatile-flow device. Following 3 months of support, the patient underwent transplantation without complications. The completion of the Fontan procedure at the time of VAD implantation, along with the use of a temporary continuous-flow device with an oxygenator, may aid in stabilizing postoperative hemodynamics. This approach could contribute to a safe transition to a durable pulsatile VAD in patients with Glenn physiology.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.529-537
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• 2005

Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.569-577
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• 1986

Between October 1979 and June 1986, modified Fontan procedures have been performed on 22 patients by the Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine. Twelve patients had tricuspid atresia; one, congenital tricuspid stenosis; five, single ventricle; two, I-transposition of the great vessels; one, double outlet right ventricle, and one, pulmonary atresia with an intact ventricular septum. There were 9 operative deaths [mortality; 40.9%]. The causes of death were right heart failure in six patients and pulmonary venous hypertension in one who misdiagnosed preoperatively. Another two were deceased due to sepsis and cerebrovascular accident at postoperative 35 and 34 days in each. There were 7 patients below 4 years of age at the time of operation and among them 4 patients were deceased. The operative death was not related with patients` age above and below 4[p=0.211]. The relation between operative death of tricuspid anomaly and another cardiac malformations was statistically significant [p=0.048]. The operative procedures with or without valved conduit [woven dacron] was related significantly [p=0.043] in the case of the 21 of the patients, but the modified Fontan operation with a valved conduit was performed early stage in this series. Since 1982, we operated on 4 patients, doing a right atrium-right ventricle anastomosis without a conduit. All survived and remained in functional class I [NYHA]. The right atrial pressure [RAP] was elevated significantly after operation [mean 9.9$\pm$4.8 ~16.9$\pm$3.6 mmHg, p<0.001]. The relation between the postoperative RAP of the survival group [16.5$\pm$4.3 mmHg] and the group who died [17.4$\pm$2.2 mmHg] was statistically significant [p=0.047]. There was no relation between any operative death and any previous palliation. All patients were followed for 4 months to 80 months, except one who was lost to follow up at 2 months following surgery [mean 11.4 months, 238 patient. months]. All were in functional class I with 5 on medications and 7 not. One was reoperated at 70 months following the first operation, due to conduit stenosis. She was moderately impaired in activity, with hepatomegaly after the second operation.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.457-462
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• 2003

Background: Surgical results of the Fontan procedures in patients with a single ventricle have improved. As the perioperative mortality continues to decline and late outcome is forthcoming, attention is now being directed toward late complications of the Fontan procedures. We retrospectively analyzed our experience with reoperations after Fontan procedures. Material and Method: Between January 1988 and December 2002, 24 patients underwent reoperations after Fontan procedures. The median age at Fontan procedures and reoperation was 3.3 years and 9.2 years, respectively. Types of initial Fontan procedures were atriopulmonary connection (n＝11), lateral tunnel Fontan (n＝11), and extracardiac conduit Fontan (n＝2). Indications for reoperation included atrioventricular valve regurgitation (n＝7), atrial arrhythmia (n＝8), Fontan pathway stenosis (n＝7), residual right-to-left shunt (n＝5), etc. Result: Procedures performed at reoperation included atrioventricular valve replacement (n＝6), conversion to lateral tunnel Fontan (n＝5), conversion to extracardiac conduit Fontan (n＝3), cryoablation of arrhythmia circuit (n＝7), etc. There was no operative mortality. There were 2 late deaths. Mean follow-up duration was 2.7$\pm$2.1 years. All patients except two were in NYHA class I at the latest follow-up. Among 8 patients with preoperative atrial arrhythmia, postoperative conversion to normal sinus rhythm was achieved in 7 patients. Conclusion: Reoperations after Fontan procedures could be achieved with low mortality and morbidity. Reoperation may lead to clinical improvement in patients with specific target conditions such as atrioventricular valve regurgitation, refractory atrial arrhythmia, or Fontan pathway stenosis, especially in patients with previous atriopulmonary connection.

• Journal of Chest Surgery
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• v.35 no.2
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• pp.137-140
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• 2002

Aortic regurgitation in the pediatrics who had previous cardiac surgery is increased with their improved longterm survival rate and their complexity of heart disease. So the need of aortic valve surgery in pediatrics is also on the increase. A 10-year old boy was admitted for progressive cyanosis and dyspnea on exertion(DOE). The patient had been underwent lateral tunnel Fontan operation before. Echocardiography and cardiac catheterization study revealed hepatic vein drained to pulmonary atrium via intrahepatic collaterals, moderate atrioventricular regurgitation, and severe aortic regurgitation due to aortic root dilation. We report a case who had aortic root reconstruction, valvuloplasty of the atrioventricular valve, and hepatic vein ligation successfully Cyanosis and DOE was dramatically improved after the operation

• Journal of Chest Surgery
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• v.35 no.6
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• pp.439-448
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• 2002

Pulmonary artery banding (PAB) in the functional univentricular heart (UVH) is a palliative procedure for staging toward the Fontan procedure; however, it is known to be a risk factor. Material and method： The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively. We investigated the aortic arch obstruction, the development and progression of subaortic stenosis after PAB, and risk factor of mortality according to surgical method. Result： In 37 neonates and infants with single ventricular physiology, aortic arch obstruction was combined in 7. There were 6 early deaths (16.2%) after PAB and 3 late deaths (8.1%) after Fontan operation. The actuarial overall survival including early mortality at 3 and 5 years were 80.7$\pm$6.6%, 72.2$\pm$8.2% respectively. Among 31 patients who survived PAB, 27 patients (87.1 %) could become candidates for Fontan operation； 22 patients (71.0%) completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS) or Fontan operation (follow-up mean 4.5 year, minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29, 27.6%)； 3 cases in the patients without arch anomaly (3/22, 13.6%) and 5 in those with arch anomaly (5/7, 71.4%). The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS) in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p＜0.001). The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). Conclusion： PAB is effective as an initial palliative step in functional UVH. And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step, with subsequent DKS for subaortic stenosis. This strategy, initial PAB and careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.

• Journal of Chest Surgery
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• v.18 no.4
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• pp.643-648
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• 1985

Tricuspid atresia is the third most common cause of cyanotic heart disease, following T.O.F. and T.G.A. It is seen in about 30% of patients with congenital heart disease on postmortem examination. Recently, we experienced a case of tricuspid atresia, who had received Blalock-Taussig Shunt operation 10 years ago. The Patient was 15 year-old girl with the complaints of persistent cyanosis and exertional dyspnea. Cineangiography revealed Keith type lib tricuspid atresia, so, RA appendage was anastomosed to the right pulmonary artery and ASD was also closed using patch. Postoperative course was very difficult because of persistent right heart failure and weak respiratory power, but from postoperative 15th day, all cardiac and respiratory problems were resolved. We followed up this patient for about 1 year, and her condition is excellent up to now.

• 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.30 no.3
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• pp.263-269
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• 1997

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1362-1369
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• 2005

Purpose : This study aimed to investigate the correlation between the plasma level of N-terminal pro-B-type natriuretic peptide(pro-BNP) and several known risk factors influencing outcomes after Fontan operations, and to assess whether pro-BNP levels can be used as predictive risk factors in Fontan operations. Methods : Plasma pro-BNP concentrations were measured in 35 patients with complex cardiac anomalies before catheterization. Cardiac catheterization was performed in all subjects. Mean right atrium pressure, mean pulmonary artery pressure(PAP), and ventricular end-diastolic pressure(EDP) were obtained. Cardiac output and pulmonary vascular resistance were calculated by Fick method. Results : Plasma pro-BNP levels exhibited statistically significant positive correlations with mean PAP(r=0.70, P<0.001), pulmonary vascular resistance(r=0.57, P<0.001), RVEDP(r=0.63, P<0.001), LVEDP(r=0.74, P<0.001), and cardiothoracic ratio(r=0.71, P<0.001). The area under the ROC curve using pro-BNP level to differentiate risk groups in Fontan operations was high : 0.868(95 percent CI, 0.712-1.023, P<0.01). The cutoff value of pro-BNP concentrations for the detection of risk groups in Fontan operations was determined to be 332.4 pg/mL(sensitivity 83.3 percent, specificity 82.7 percent). Conclusion : These data suggest that plasma pro-BNP levels may be used as a predictive risk factor in Fontan operations, and as a guide to determine the mode of therapy during follow-up after Fontan operations.