• Journal of Chest Surgery
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• v.51 no.2
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• pp.130-132
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• 2018

Coronary sinus ostial atresia (CSOA) with persistent left superior vena cava (LSVC) in the absence of an unroofed coronary sinus is a benign and rare anomaly that may be taken lightly in most instances. However, if overlooked in patients undergoing univentricular heart repair such as bidirectional Glenn or Fontan-type surgery, fatal surgical outcomes may occur due to coronary venous drainage failure. We report a case of CSOA with a persistent LSVC that was managed through coronary sinus rerouting during a total cavopulmonary connection, and provide a review of the literature regarding this rare anomaly.

• 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.43 no.4
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• pp.364-374
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• 2010

Background: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. Material and Method: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, $11.7{\pm}13.2days$) and weight at operation ranged from 2.2 to 4.8 kg (mean, $3.17{\pm}0.52kg$). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean $26.0{\pm}22.8months$). Result: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. Conclusion: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.16-21
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• 1990

From November 1978 through June 1989, 33 patients aged 3 months to 27 years [mean 9.7 years] underwent repair of intracardiac defects associated with corrected transposition. Five patients had had previous palliative surgery. Operation were performed in 31 for ventricular septal defect, 22 for pulmonary outflow tract obstruction, 16 for atrial septal defect, and 5 for anatomical tricuspid valve regurgitation. Pulmonary outflow tract obstruction was relieved by pulmonary valvotomy in 9, Rastelli procedure in 5, modified Fontan procedure in 3, and by REV procedure in 5 patients recently. Early mortality was 21.2%[7/33] and no late mortality during follow up period. Two had residual pulmonary outflow tract obstruction and one residual VSD. In eight patients, transient arrhythmia was found but soon returned to sinus rhythm. Five patients developed complete heart block and 2 were given permanent pacemaker insertion. There were 8 RBBB, 1 LBBB and one second degree atrioventricular block patients, but all showed no clinical significance. This report suggests that surgical repair of intracardiac defects associated with corrected transposition can be achieved with acceptable low risk. Though the mortality is still high, we can improved the result by advancing surgical technique, knowledge of the special conduction system, and by improving postoperative care.

• Journal of Chest Surgery
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• v.47 no.4
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• pp.344-349
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• 2014

Background: The Damus-Kaye-Stansel (DKS) procedure is a method for mitigating the risk of systemic ventricular outflow tract obstruction (SVOTO). However, there have been few reports on which surgical technique shows a better outcome. The objective of this study was to compare the outcome of the DKS procedure according to the surgical technique used. Methods: We retrospectively reviewed 12 consecutive patients who underwent the DKS procedure from March 2004 to April 2013. When the relationship of the great arteries was anterior-posterior, the double-barrel technique (group A) was performed. If the relationship was side-by-side, the ascending aortic flap technique (group B) was performed. Results: There was no early mortality and 1 late mortality in group B. There was no statistically significant difference in the median peak pressure gradient of preoperative subaortic stenosis in both groups: 14 mmHg (range, 4 to 53 mmHg) in group A and 15 mmHg (range, 0 to 30 mmHg) in group B (p=0.526). Further, a significant postoperative pressure gradient was not observed in either group A or group B. More than moderate postoperative neoaortic regurgitation was observed in 1 patient of group B; this patient underwent neoaortic valve replacement 66 months after the DKS procedure. No one had a recurrent SVOTO during follow-up. Conclusion: The DKS procedure is an effective way to minimize the risk of SVOTO, and there is little difference in the outcomes of the DKS procedure according to the surgical technique used.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.618-626
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• 1986

Univentricular heart is a rare congenital cardiac anomaly in which the atrial chambers are connected to only one ventricular chamber and it consists of a diverse group of cardiac malformation characterized by both AV valves or a common AV valve opening into the same ventricle, or the presence of only a solitary AV valve. In spite of recent development in cardiac surgery, corrective operations for univentricular heart still have high mortality and complication rate. Twenty eight patients underwent corrective operation for univentricular heart at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital from February 1979 to July 1986. Of the 28 patients, 7 patients were operated on by ventricular septation and 21 patients by modified Fontan operation. Of the 28 patients, 19 patients were male and 9 patients female and ages ranged from 5 months to 18 years old with the average age of 7.3 years. There were 2 mortalities in 7 patients operated on by septation with the mortality rate of 28.6% and 5 complications, 3 complete AV block, 1 low cardiac output and 1 arrhythmia. All survived patients are being followed up without specific problem till now. There were 10 mortalities in 21 patients operated on by modified Fontan operation with the mortality rate of 47.6% and 10 complications, 2 low cardiac output, 2 respiratory failure necessitating tracheostomy, 2 persistent cyanosis, 2 arrhythmia, 1 missing of left AV valve in situs inversus patient due to misdiagnosis and one rupture of closed right AV valve. Incremental risk factors for operative mortality are young age less than 5 years old, anomalous pulmonary and systemic venous drainage and atrial septation procedure. In 11 survived patients, 9 patients show good follow-up results but one patient complains of persistent cyanosis and another one patient is suffered from CHF. In our series, results of corrective operation for univentricular heart shows continuing improvement but still high mortality and complication rate. So there must be continuing improvement in surgical result by selection of patient, by adequate decision making for timing and method of operation and by improving operative methods.

• 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.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.1-11
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• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

• Journal of Chest Surgery
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• v.50 no.3
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• pp.215-219
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• 2017

Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. PAVFs were present in the rig ht lung, and rig ht lung perfusion was nearly absent. After coil embolization, takedown of the Glenn shunt, and reconstruction of the rig ht pulmonary artery, the patient's symptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.

• Journal of Chest Surgery
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• v.25 no.12
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• pp.1542-1549
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• 1992

From March 1986 to August 1992, 18 patients underwent diaphragmatic plication for the diaphragmatic paralyses complicating various pediatric cardiac procedures. Age at operation ranged from 16 day to 84 months with mean age of 11.8 months. In order of decreasing incidence, the primary cardiac procedures included modified Blalock-Taussig shunt [ 5 ], Arterial switch operation [ 4 ], modified Fontan operation [ 2 ], and others [ 7 ]. The suspicious causes of phrenic nerve injury included overzealous pericardial resection [ 7 ], direct trauma during the procedure [ 6 ], dissection of fibrous adhesion around the phrenic nerve [ 3 ] and unknown etiology [ 2 ]. The involved sides of diaphragm were right in 10, left in 7 and bilateral in one. The diagnosis was suspected by the elevation of hem-idiaphragm on chest x-ray and confirmed by fluoroscopy. The interval between primary operation and plication ranged from the day of operation to 38 postoperative days [mean : 14 days]. The method of plication were "Central pleating technique" described by Schwartz in 16 and other techniques in 2. Five patients expired after plication and the cause of death were not thought to be correlated directly with the plication itself. In the remaining 13 survivors, extubation or cessation of positive ventilation could be done between the periods of the day of plication and 14th postoperative days [mean; 3.8day]. We have made the following conclusions : 1] Phrenic nerve paralyses are relatively common complication after pediatric cardiac procedures and the causes of phrenic nerve injury are mostly preventable; 2] Phrenic nerve palsy is associated with corisiderable morbidity; 3] diaphragmatic plication is safe, reliable and can be applicable in patients who are younger age and require prolonged positive pressure ventilation.ntilation.