• Journal of Chest Surgery
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• v.42 no.6
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• pp.770-773
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• 2009

Rhabdomyoma has been reported to be the most common type of cardiac tumors in fetuses and children, and multiple cardiac rhabdomyomas almost certainly signify the association with tuberous sclerosis. We report here on a case of Tetralogy of Fallot (ToF) that was associated with multiple rhabdomyomas and tuberous sclerosis. A two-year-old boy, who had undergone systemic-pulmonary shunt during the neonatal period, received total correction of his ToF after the complete regression of the cardiac tumor. The postoperative course was uneventful, and he has been follow-up for 2 months. The boy is currently in an excellent condition.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.115-122
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• 1991

From April 1986 to December 1989, 25 infants under the age of 12 months with tetralogy of Fallot were operated on. Age ranged from 3 to 12 months[mean 8.9$\pm$4.9 months] and mean body weight was 7.8$\pm$ 2.6kg. All the patients were deeply cyanotic, 12 of them experienced anoxic spell. Transannular patch was laid down in 19 patients, in 7 of them monocuspid patch was utilized. Postrepair P RV/LV was measured at operation room in 17 patients[mean 0.48$\pm$0. 14]. Hospital mortality was 20Yo. Causes of deaths include right ventricular failure and low cardiac output. The mortality was closely related with patient`s age and body surface area at operation. Also higher mortality was noticed in patients having major associated anomaly or previous palliative operation, preoperative management with propranolol and transannular repair. 18 patients were followed up for 12 to 50 months with a mean follow-up time of 24 months after operation. There were no late deaths and late ventricular arrhythmia or congestive heart failure was not detected as yet. Redo operation was performed in one case because of residual pulmonic stenosis. Considering several advantages of early primary repair, primary repair of symptomatic infants with tetralogy of Fallot should be encouraged despite somewhat high mortality rate as yet and better results could be anticipated along with improvement of myocardial protection method and postoperative care.

• 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.23 no.2
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• pp.253-259
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• 1990

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.153-160
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• 1991

Tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 91 of 250 patients treated surgically [including redo operations] between April 1986 and December 1989. Their age ranged from 6 months to 14 years [mean 39.7 months]. Associated cardiovascular anomalies were right aortic arch [n=22], ASD [n=12], PDA [n=5], persistent left SVC [n=5], and others [n=6]. PA index was measured pre-operatively since 1987 to estimate pulmonary artery size and safe total correction[mean 289$\pm$110mm2/BSA]. Eight patients received previous shunt take down procedure concomitantly. Pulmonary arteriotomy was extended through small pulmonary annulus to a minimal distance upon the right ventricular infundibulum and transannular patch was applied in 38 patients [41.3%], in 31 of them monocuspid patch was utilized. pRV/LV was measured at operation room in 77 patients [mean 0.58$\pm$0.36]. Operative mortality was 6.6% [6/91]. The causes of death were low cardiac output [n=5], arrhythmia[n=1] and respiratory failure [n=1]. At follow-up between 12 months and 57 months [mean 30.8 months] most patients were in New York Heart Association class I without cardiac medication. There was no late death, but reoperations were required in 3 patients to relieve residual right ventricular outflow obstruction. Thus successful repair of tetralogy of Fallot can be accomplished in most patients including infants by transatrial-transpulmonary approach and the better result can be anticipated with respect to postoperative right ventricular function and arrhythmia than the conventional transventricular approach.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.337-341
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• 1983

The experience with operative treatment for total correction of Tetralogy of Fallot at the department of Thoracic and Cardiovascular Surgery, Korea University Hospital from January, 1977, through April, 1983 was reviewed. Of the 29 patients reviewed, male to female occurrence ratio was 22:7 Type of V.S.D. was type II in 26 cases[90%] and total conus defect in 3 cases[10%]. Average size of V.S.D. was 19 mm. Type of Right ventricular outflow tract stenosis was highest frequency with pulmonary valvular and infundibular stenosis combined type in 21 cases[72%], and there were 8 deaths In this group. Type of R.V.O.T. reconstruction contains 2 cases of infundibulectomy only, 1 case of infundibulectomy with valvular commissurotomy, 2 cases of pericardial patch, 23 Cases of pericardial patch with Teflon or Dacron felt reinforced and 1 case of pulmonary valved conduit reconstruction. Operative mortality was higher in outflow patch through pulmonary valve ring. Overall mortality was 31%. Major causes of death and postoperative complications were low output syndrome, complete A-V block acute renal failure, ventricular fibrillation, bleeding brain abscess, and sudden cardiac arrest.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.460-462
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• 2002

We experienced primary repair of complete atrioventricular septal defect with Teralogy of Fallot. The diagnosis was established preoperatively by echocardiography, cardiac catheterization and cardioangiography. Repair was accomplished using cardiopulmonary bypass. Two patch techinque were performed using Dacron patch for ventricular septal defect and pericardial patch for atrial septal defect. Infundibullectomy and right ventricular outflow tract reconstruction with the transannular pericardial patch were performed. The postoperative echocardiography showed mild mitral and tricuspid regurgitation, but there were no hemodynamic abnormalities.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1197-1201
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• 1996

From October, 1987 to December, 1995, nine patients underwent total correction of tetralogy of Fallot in adults over 20 years of age. There were 5 male and 4 female patients aged from 22 to 42 years(mean, 29.6 years). Three patients were in New York Heart Association(NYHA) functional class II, and 6 patients in class III. The hemoglobin values ranged from 10.8 to 20.7 gm/㎗ (average, l5.6gm/㎗). The preoperative clinical features were as follows: cyanosis, 8 patients; dyspnea on exertion, 6 patients; clubbing of fingers, 5 patients; frequent upper respiratory infection, 3 patients. At the operation both infundibular and valvular stenosis were present in all patients. Reconstruction of right ventricular outflow tract(RVOT) using Goretex was required in 7 patients, and transannular patching with Goretex in 2 pateints. Left pulmonary angioplasty with pericardium was done in 2 patients. No hospital deaths occurred. Four of 9 patients(44.4%) had postoperative low cardiac output syndrome, and postoperative bleeding in 5. One patient required reoperation due to residual ventricular septal defect and tricuspid regurgitation 3 months after the first operation. The mean follow-up period was 25 months, range 11 to 77 months. All was asymptomatic and in NYHA class I. We suggest that advanced age is not contraindication to surgery in tetralogy of Fallot, and tetralogy of Fallot in adults could be operated on due to low mortality.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.40-48
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• 1983

Tetralogy of Fallot is characterized by the interventricular septal defect associated with obstruction of the right ventricular outflow. The importance of the latter anatomic malformation was clinically evaluated according to the angiographic measurements of the pulmonary arteries along with the pulmonary valve annulus. Seventy of a total 76 patients operated on during a whole year of 1981 were the patients for clinical evaluation. Fifteen patients died within 1 month after operation with the operative mortality of 21.4%. The young age and the severity of pulmonary arterial hypoplasia were ones among the surgical risk factors at a total corrective surgery of tetralogy. Preoperative angiographic measurements of the pulmonary arteries to speculate the expected postoperative ratio between the left ventricular and the right ventricular pressures were retrospectively calculated according to the formula. The predicted values of P RV/LV greater than 0.5 carried apparenliy higher complication and mortality rates than the group of P RV/LV less then 0.5. The selection of the candidates for either a total correction or the staged operation In tetralogy of Fallot can be evaluated on the preoperative angiographic measurements and the expected Improvements of the clinical results were discussed.

• Journal of Chest Surgery
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• v.8 no.1
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• pp.61-64
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• 1975