• Clinical and Experimental Pediatrics
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• v.57 no.1
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• pp.19-25
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• 2014

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.

• Clinical and Experimental Pediatrics
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• v.56 no.6
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• pp.235-241
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• 2013

Repair of tetralogy of Fallot (TOF) has shown excellent outcomes. However it leaves varying degrees of residual hemodynamic impairment, with severe pulmonary stenosis (PS) and free pulmonary regurgitation (PR) at both ends of the spectrum. Since the 1980s, studies evaluating late outcomes after TOF repair revealed the adverse impacts of residual chronic PR on RV volume and function; thus, a turnaround of operational strategies has occurred from aggressive RV outflow tract (RVOT) reconstruction for complete relief of RVOT obstruction to conservative RVOT reconstruction for limiting PR. This transformation has raised the question of how much residual PS after conservative RVOT reconstruction is acceptable. Besides, as pulmonary valve replacement (PVR) increases in patients with RV deterioration from residual PR, there is concern regarding when it should be performed. Regarding residual PS, several studies revealed that PS in addition to PR was associated with less PR and a small RV volume. This suggests that PS combined with PR makes RV diastolic property to protect against dilatation through RV hypertrophy and supports conservative RVOT enlargement despite residual PS. Also, several studies have revealed the pre-PVR threshold of RV parameters for the normalization of RV volume and function after PVR, and based on these results, the indications for PVR have been revised. Although there is no established strategy, better understanding of RV mechanics, development of new surgical and interventional techniques, and evidence for the effect of PVR on RV reverse remodeling and its late outcome will aid us to optimize the management of TOF.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.780-785
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• 1987

Pulmonary atresia with VSD is uncommon congenital anomaly with high mortality in neonatal period. Recently we experienced surgical correction of 2 cases of pulmonary atresia with VSD. The first case was 7-year old female patient and diagnosed as pulmonary atresia with VSD combined PDA. So, total correction was undertaken which consisted of PDA ligation, patch repair of VSD, transannular enlargement of RVOT with woven Dacron vascular graft, and closure of PFO. Postoperative systemic Rt. ventricular and radial artery pressure ratio was 0.44 and her postoperative course was uneventful. The second case was 6-year old male patient diagnosed as pulmonary atresia with VSD and large systemic-pulmonary collateral arteries. There were two large systemic-pulmonary collaterals, one was simply controlled by ligation, but the other was considered to supply Rt. upper lung. So end to side anastomosis was performed to the RVOT patch. Postoperative systolic Rt. ventricular and radial artery pressure ratio was 0.54. During the follow up period he showed clinical picture of Rt. heart failure, which is relatively well controlled with anticongestive therapy.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.250-257
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• 1985

Despite low mortality and excellent long-term results after repair of Tetralogy of Fallot, certain associated anomaly such as single pulmonary artery continues to be told to have a high operative mortality and morbidity, and there is still some debate on appropriate surgical intervention. During the 4 year period from 1981 to 1984, surgical repair was performed on 5 patients with tetralogy of Fallot and congenital [4 cases] or acquired [1 case] absence of left pulmonary artery. Previous left pneumonectomy had been performed in the patient with acquired absence of a pulmonary artery. Transannular patch or RVOT patch alone with or without pulmonic valvotomy was used with some modifications to reduce pulmonic insufficiency in individual patient. In contrast with previous reports, all patients survived operation and have exhibited marked symptomatic improvement without pulmonic valve insertion nor valved conduit.

• Journal of Chest Surgery
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• v.27 no.6
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• pp.435-443
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• 1994

Surgical procedures to relieve congenital right ventricular outflow tract[RVOT] obstruction of heart were performed on 125 patients from September 1985 to August 1992. There were 65 males and 60 females. Ages ranged from 7 months to 33 years with a mean age of 8 years. All the patients were divided into three main groups[I, II, III] depending on the presence or absence of cyanosis and combined anomalies. The patient were classified into two groups; A and B according to the outcome after surgical repair. Group A included the patients who had a good postoperative outcome with or without mild complications such as wound disruption, or hydrothorax. Group B included the patients who had a poor outcome including hospital death and significant postoperative complications such as heart failure, low output syndrome, respiratory failure, hepatic failure and others. And the results were summarized as follows. 1. There were no significant differences in age, body surface area and aortic dimension among the group I, II, and III, but there were significant differences among groups in pulmonary arterial dimension, ACT[aortic cross clamping time], TBT [total bypass time], preoperative and postoperative ratio of systolic pressure of right and left ventricles [pre PRV/RV and post PRV/LV], and the size of Hegar dilator which passed through the RVOT postoperatively [p<0.05]. 2. In the group A and B, there were significant differences in pulmonary arterial dimension [group A:1.6$\pm$0.5 cm, group B:1.9$\pm$0.6 cm], ACT [group A:102.3$\pm$ 46.0 minute, group B:76.1$\pm$46.1 minute], TBT [group A:133.9$\pm$56.6 minute, group B:94.9$\pm$51.9 minute], pre PRV/LV [group A:1.06$\pm$0.24, group B:0.8$\pm$0.32], post PRV/LV [group A:0.58$\pm$0.18, group B:0.43$\pm$0.16].It has been concluded that postoperative prognosis of RVOT obstruction was influenced by pulmonary arterial dimension, ACT, TBT, severity of RVOT obstruction [pre PRV/LV] and post PRV/LV.

• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.856-859
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• 2005

A male neonate was referred to our hospital with facial cyanosis and tachypnea at 19 days of age. Two-dimensional echocardiography showed type B interrupted aortic arch, posterior malalignment ventricular septal defect and valvular aortic stenosis. A new surgical repair was done with biventricular repair and neo-aortic arch reconstruction. Left ventricular outflow track (LVOT) was consisted of aortic valve and pulmonic valve. Right ventricular outflow (RVOT) track was reconstructed with extracardiac conduit. Postoperative two-dimensional echocardiography showed no stenosis and turbulency flow on LVOT and RVOT.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.250-258
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• 2003

Purpose : The actual clinical examples of co-appliance of catheter intervention with surgical procedures in the treatment of pulmonary atresia with an intact ventricular septum(PA/IVS) which we have experienced in our institution are here shown, and the anatomical and hemodynamical profiles between each method is compared. Methods : Medical records of 33 patients with PA/IVS who underwent various treatment from January, 1995 to December, 2000 were reviewed for a retrograde study. Results : In three out of 10 patients who underwent percutaneous balloon pulmonary valvotomy (PPV), residual pulmonary stenosis were observed in their out patient department(OPD) follow-ups, eventually necessitatig balloon pulmonary valvuloplasty(BPV). One out of three patients exhibited deterioration of tricuspid regurgitation after BPV, requiring surgical tricuspid annuloplasty(TAP). Two out of the seven patients who received primarily surgical right ventricle outlet tract(RVOT) repair without any systemic-pulmonary shunt or intervention needed additional intervention employing cardiac catheterization after operation. Two patients received interventional catheterization before surgical RVOT repair. In five out of 11 cases of Fontan type operation, coil embolization of collateral circulation was done before total cavo-pulmonary connection(TCPC), and in three cases, interventional catheterization was needed after TCPC. Conclusion : Both medical and surgical treatment modalities are widely used in management of PA/IVS patients, and recent results prove that medico-surgical cooperative treatment is essential.

• Journal of Chest Surgery
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• v.44 no.2
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• pp.108-114
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• 2011

Background: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. Materials and Methods: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was $79.2{\pm}14.8$ months. Results: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. Conclusion: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.588-597
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• 2006

Background: The surgical repair of a tetralogy of Fallot (TOF) has been performed successfully with a favorable early and late outcome. However, the later development of pulmonary regurgitation and stenosis remains a problem. The development of pulmonary regurgitation and stenosis may be changed by the size of right ventricular outflow tract (RVOT) reconstruction at the initial total correction. Hence, it is necessary to investigate the ideal size of RVOT reconstruction. Material and Method: This prospective study was carried out to determine how a surgical strategy and the RVOT size affect the occurrence of pulmonary regurgitation and stenosis. From January 2002 to December 2004, 62 patients underwent the TOF total correction. The RVOT size (diameter of pulmonary valve annulus) of each case was measured after the RVOT reconstruction and converted to a Z value. A pre-scheduled follow up (at discharge, 6 months, 1 year, 2 years and 3 years) was carried out by echocardiography to evaluate the level of pulmonary regurgitation and stenosis. Result: The patients were divided to two groups (transannular group n=12, nontransannular group n=50) according to the method of a RVOT reconstruction. The Z value of RVOT=iameter of pulmonary valve annulus) (transannular group -1, $range\;-3.6{\sim}-0.8;$ nontransannular group -2.1, $range\;-5.2{\sim}-1.5)$ and the average pRV/LV after surgery ${(transannular group 0.44{\pm}0.09,\;nontransannular group\;0.42{\pm}0.09)}$ did not show any significant difference between two groups. The occurrence of pulmonary regurgitation above a moderate degree was more frequent in the transannular group (p<0.01). In nontransannular group, the development of pulmonary regurgitation more than moderate degree occurred to the patients with larger RVOT size (Z value>0, p<0.02) and the progressing pulmonary stenosis more than mild to moderate degree developed in the patients with smaller RVOT size (Z value<-1.5, p<0.05). A moderate degree of pulmonary stenosis developed for 4 nontransannular patients. Three underwent additional surgery and one underwent a balloon valvuloplasty. Their Z value of RVOT were -3.8, -3.8 -2.9, -1.8, respectively. Conclusion: When carring out a TOF total correction, transannular RVOT reconstruction group has significantly more pulmonary regurgitation. In the nontransannular RVOT reconstruction. the size of the RVOT should be maintained from Z value -1.5 to 0. If the Z value is less than -1.5, we should follow up carefully for the possibility of pulmonary stenosis.

• Journal of Chest Surgery
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• v.17 no.4
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• pp.632-635
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• 1984

Hypoplastic RV was rarely combined with various other intracardiac anomalies. We experienced the excellent result after surgical correction in these 2 patients. 1. F/7: Combined anomalies were, [1] Hypoplastic Rt. Coronary art.[2] Hypoplastic RV [3] ASD [secundum] & [4] PS [infundibular & valvular] After CP Bypass, [1] Direct suture of ASD [2] Infundibulectomy [3] Pul valvulotomy & [4] Patch enlargement of RVOT with Pericardial monocusp were done 2. F/14: Combined anomalies were, [1] Hypoplastic RV [2] P5 [infundibular] [3] T5 [4] VSD [Type II] [5] ASD [secundum] & [6] 2\ulcornerA-V Block fter CP Bypass, [1] Infundibulectomy [2] Tricuspid valvulotomy [3] Patch repair of VSD & ASD & [4] Implantation of permanent pacemaker were done. Above operations resulted in marked improvement with disappearance of cyanosis & dyspnea.