• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.269-274
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• 2006

Background: Tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAS) is complex lesion with marked heterogeneity of pulmonary blood supply and arborization anomalies. Patients with TOF with PA and MAPCAS have traditionally required multiple staged unifocalization of pulmonary blood supply before undergoing complete repair. In this report, we describe recent change of strategy and the results in our institution. Material and Method: We established surgical stratagies: early correction, central mediastinal approach, initial RV-PA conduit interposition, and aggressive intervention. Between July 1998 and August 2004, 23 patients were surgically treated at our institution. We divided them into 3 groups by initial operation method; group I: one stage total correction, group II: RV-PA conduit and unifocalization, group III: RV-PA conduit interposition only. Result: Mean ages at initial operation in each group were $13.9{\pm}16.0$ months (group 1), $10.4{\pm}15.6$ months (group II), and $7.9{\pm}7.7$ months (group III). True pulmonary arteries were not present in f patient and the pulmonary arteries were confluent in 22 patients. The balloon angioplasty was done in average 1.3 times (range: $1{\sim}6$). There were 4 early deaths relating initial operation, and 1 late death due to incracranial hemorrhage after definitive repair. The operative mortalities of initial procedures in each group were 25.0% (1/4: group I), 20.0% (2/10: group II), and 12.2% (1/9: group III). The causes of operative mortality were hypoxia (2), low cardiac output (1) and sudden cardiac arrest (1). Definitive repair rates in each group were 75% (3/4) in group I, 20% (2/10, fenestration: 2) in group II, and 55.0% (5/9, fenestration: 1) in group III. Conclusion: In patients of TOF with PA and MAPCAS, RV-PA connection as a initial procedure could be performed with relatively low risk, and high rate of definitive repair can be obtained in the help of balloon pulmonary angioplasty. One stage RV-PA connection and unifocalization appeared to be successful in selected patients.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.259-264
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• 2003

Purpose : We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. Methods : We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. Results : No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. Conclusion : In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.48-51
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• 2002

The conversion of failing Fontan circuit to total cavopulmonary connection(TCPC) is recommended as a therapeutic option in patients with late Fontan complications such as atrial arrhythmia, atrial enlargement, pulmonary venous obstruction, and ventricular dysfunction. Combined TCPC with extracardiac conduit and cryoablation of arrhythmia circuit is preferred for treatment of failing Fontan coulection with atrial lachyarrhythrnia. We report a case of conversion of atriopulmonary connection to extracardiac conduit Fontan and cryoablation of atrial arrhythmia circuit in a patient with tricuspid atresia, who also had ectopic atrial tachycardia, right atrial thrombi, pulmonary venous obstruction, and ventricular dysfunction. This patient and the parents were Jehovah's Witnesses; therefore, the patient underwent the procedure without blood transfusion.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.303-306
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• 2002

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.277-283
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• 2005

Arterial oxygen saturation $(SaO_2)$ instability frequently takes place after systemic-pulmonary shunt without shunt occlusion. We analyzed actual incidence and risk factors for $SaO_2$ instability after shunt operations, and possible mechanisms were speculated on. Material and Method: Ninety three patients, who underwent modified Blalock-Taussig shunt from January 1996 to December 2000, were enrolled in this study. Adequacy of shunt was verified in all patients, either by ensuing one ventricle or biventricular repair later on or by appropriate pulmonary artery growth on postoperative angiogram. Age, body weight, hemoglobin level at operation were 3 day to 36 years (median: 1.8 months), 2.5kg to 51kg (median: 4.1kg) and $10.7\~24.3$ gm/dL (median: 15.2 gm/dL) respectively. Preoperative diagnoses were functional single ventricle with pulmonary stenosis or atresia in 39, tetralogy of Fallot in 38 and pulmonary atresia with intact ventricular septum in 16. Pulmonary blood flow (PBF) was maintained pre-operatively by patent ductus or previous shunt in 64 and by forward flow through stenotic right ventricular outflow tract (RVOT) in 29. $SaO_2$ instability was defined as $SaO_2$ less than $50\%$ for more than 1 hour with neither anatomic obstruction of shunt nor respiratory problem. Result: 10 patients $(10.7\%)$ showed $SaO_2$ instability after shunt operation. After shunt occlusion was ruled out by echocardiogram, they received measures to lower pulmonary vascular resistance (PVR), which worked within a few hours in all patients. Risk factors for $SaO_2$ instability included older age at operation (p=0.039), lower preoperative $SaO_2$ (p=0.0001) and emergency operation (p=0.001). PBF through stenotic RVOT showed marginal statistical significance (p=0.065). Conclusion: $SaO_2$ instability occurs frequently after shunt operation, especially in patients with severe hypoxia pre-operatively or unstable clinical condition necessitating emergency operation. Temporary elevation of pulmonary vascular resistance is a possible mechanism in this specific clinical setting.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.635-642
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• 2006

Purpose : The purpose of this study is to determine whether the new pulmonary valve reconstruction technique prevents short-term postoperative pulmonary regurgitation and improves early and mid-term clinical outcome. Methods : We reviewed postoperative echocardiographic variables and chest X-ray films from 31 patients who had undergone valve reconstruction(pulmonary valve reconstruction group : PVR) for the repair of TOF between April 2000 and August 2004. We compared the clinical data of these patients with those from 47 patients who had right ventricular outflow tract reconstruction with a monocusp valve(monocusp ventricular outflow patch group : MVOP) and 22 patients who had a transannular patch repair without a monocusp valve(transannular patch group : TAP). Results : In the PVR group, 25 patients(81 percent) had trivial or mild pulmonary regurgitation in their early post operative echocardiogram. Only 12 patients(26 percent) in the MVOP group had mild pulmonary regurgitation; and no patient in the TAP group had it. Pulmonary valve function was good in 96 percent of the PVR group, 36 percent of the MVOP group, and none in the TAP group in early post-operative echocardiogram. Follow-up echocardiogram(1, 2, 3, 4 years later) of the MVOP and TAP groups showed moderate pulmonary regurgitation and severely decreased valve function in almost all cases. However, in the PVR group 54 percent(16/28), 50 percent(14/28), 37 percent(9/24), and 31 percent(5/16) of the patients had trivial or mild pulmonary regurgitation 1, 2, 3 and 4 years after operation, respectively. The valve function remained good in 80 percent(24/30), 64 percent(18/28), 57 percent(12/21), and 31 percent(5/16) of the patients 1, 2, 3 and 4 years after operation respectively. Conclusion : Pulmonary valve reconstruction is effective in reducing pulmonary regurgitation and right ventricular dilatation in the repair of TOF, even though regurgitation increases with time. Further study is needed to determine long-term results.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.462-468
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• 2007

Purpose : The purpose of this study was to analyze the changes of the late potential of Signal- averaged electrocardiography (SAECG) and Electrocardiography (ECG) parameters during follow up of those who had taken surgical repair of Tetralogy of Fallot (TOF). Methods : Nine patients who had taken surgical repair of TOF since 1985 checked SAECG and standard 12 leads ECG twice in 1999 and 2005 in Kyungpook national university hospital. We evaluated changes of QRS duration, QT interval and JT interval, QRS dispersion, QT dispersion and JT dispersion of standard 12 leads ECG and Filtered QRS (f-QRS), High frequency low amplitude potential (HFLA), Root mean square in terminal 40 ms (RMS) and Mean voltage in terminal 40 ms (MV) of SAECG between in 1999 and 2005. Results : There were significant decrease of JT dispersion ($101.11{\pm}50.11$ vs $71.11{\pm}22.61ms$, P< 0.05) and significant increase of HFLA ($24.67{\pm}13.19$ vs $32.89{\pm}14.21ms$, P<0.05). But there were no significant changes in other parameters. Conclusion : In repaired TOF patients, we evaluated ECG and SAECG to detect possible late complications such as tricuspid regurgitation, right ventricular enlargement, ventricualar arrythmia and sudden death. And there were significant changes of ECG and SAECG in some parameters (JT dispersion, HFLA). But to see the relationships between the changes of these parameters and the long term prognosis, we need to check more patients and longer follow-up.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.766-771
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• 2003

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. Truncus arteriosus is a rare congenital anomaly that has an unfavorable natural course. We report a successful surgical correction of truncus arteriosus in an 13-day-old premature infant with body weight of 1.5 kg and gestational age of 32 weeks. We used autologous untreated pericardial conduit without valve in right ventricular outflow reconstruction. The patients remains in good condition with normal body weight (50 percentile) and wide right ventricular outflow tract 20 months after the operation.

• Journal of Chest Surgery
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• v.36 no.10
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• pp.759-765
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• 2003

Truncus arteriosus with interrupted aortic arch is a very rare congenital cardiac anomaly that has an unfavorable natural course. We report a successful one-stage repair of truncus arteriosus with interrupted aortic arch through median sternotomy in a 25-day-old neonate weighing 3.1 kg. We reconstructed the aortic arch with direct side-to-end anastomosis between ascending and descending aortas. The right ventricular outflow reconstruction was performed with untreated autologous pericardial conduit without valve following Lecompte maneuver. The patient has been grown-up in good condition (25 ∼ 50 percentile of body weight) and shows the right ventricular outflow tract wide 1 year after the operation.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.64-67
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• 2002

Eisenmenger's syndrome is the disease of right to left shunt developing from the increased pulmonary vascular resistance caused by excessive pulmonary blood flow in patients with abnormal connections of systemic to pulmonary blood passage. The heart-lung transplantation was the only curative method in early transplantation period, but good results after bilateral lung transplantation have been reported as the fact that right heart function improved by only lung transplantation. We successfully carried out bilateral sequential single lung transplantation in a 34-year-old female patient with Eisenmenger's syndrome with large PDA. We report this case with a brief review of the literature.