• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.271-276
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• 2005

We analysed differences in operative methods and postoperative outcome according to the severity of preoperative cyanosis in adult ToF (Tetralogy of Fallot) patients. Material and Method: From August 1989 to June 2001, thirty three adult patients, 18 females and 15 males, underwent total correction for ToF. Their age ranged from 15 years to 54 years (median: 34). Patients were divided into 2 groups by preoperative $SaO_2$ (arterial oxygen saturation): group I$(n=cyanotic,\;SaO_2\;\geq94\%)$ and group II $(acyanotic,\; SaO_2\geq95%)$. Preoperative median hemoglobin level was higher in group I compared to group II (17.5 g/dl vs 15 g/dl). Postoperative follow-up duration ranged from 1 to 94 months (670 patient-month, median: 14 months), and 63 two-dimensional echocardiographic examinations were done during this period. Result: There were no early or late mortality. With regard to RVOT (right ventricular outflow tract) reconstruction, trans-annular patch and RV-PA extracardiac conduit were used in 7 and 3 patients respectively, and all of them belonged to group I. In group I, cardiopulmonary bypass time, aortic cross-clamping time, ICU day, hospital day were significantly longer than in group II, and postoperative inotropic support was significantly greater than in group II. There was no ventricular arrhythmia in both groups, and one patient in group I suffered from atrial arrhythmia, which was resolved spontaneously after tricuspid and pulmonary valve replacement. During follow-up periods, functional class, residual RVOT stenosis and pulmonary regurgitation, tricuspid regurgitation, occurrence of ventricular and atrial arrhythmias were comparable between two groups. Conclusion: In adult ToF patients with severe preoperative cyanosis, more aggressive RVOT reconstruction and careful postoperative care are mandatory. However intermediate-term outcome of this group of patients is comparable to the patients with minimal or no preoperative cyanosis.

• Journal of Chest Surgery
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• v.31 no.4
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• pp.393-397
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• 1998

A 25-year-old man with viral cardiomyopathy and chronic active hepatitis successfully underwent dynamic cardiomyoplasty for the first time in Korea on July 30, 1996. The patient had been intermittently dyspneic for 5 years and was admitted to our center twice because of heart failure. For the past 2 years, he was NYHA functional class III status with a left ventricular ejection fraction(LVEF) of around 30%. The patient was born with scoliosis and showed a short stature. The liver function showed elevated liver enzymes, and hepatitis B antigen was positive. The liver biopsy revealed chronic active hepatitis. The preoperative echocardiogram showed decreased left ventricular function with grade II mitral and grade II tricuspid regurgitation with dilated left and right atrium. Recently his symptoms worsened and we decided to perform a dynamic cardiomyoplasty. The left latissmus dorsi muscle(LDM) was mobilized and tested with lead placement on his right lateral decubitus position. The patient was positioned into supine and, after median sternotomy, the heart was wrapped with the mobilized muscle. The Russian made cardiomyostimulator(EKS-445) and leads (Myocardial PEMB for heart and PEMP-1 for LDM) were used. The total operation time was 8 hours and there were no perioperative episodes. Postoperatively the LDM had been trained for a 10 week period and currently the stimulation ratio is maintained at 1:4. The postoperative LVEF did not increase with the value of 30-35%. However, the patient feels better postoperatively with slightly increased activity.

• Tuberculosis and Respiratory Diseases
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• v.46 no.6
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• pp.846-855
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• 1999

Background : Secondary pulmonary hypertension is an important final endpoint in patients with chronic hypoxic lung disease, accompanied by deterioration of pulmonary hemodynamics. The clinical diagnosis of pulmonary hypertension and/or cor pulmonale could be difficult, and simple noninvasive evaluation of pulmonary artery pressures has been an relevant clinical challenge for many years. Doppler echocardiography might to be a more reliable method for evaluating pulmonary hemodynamics in such patients in terms of the accuracy, reproducibility and easiness for obtaining an appropriate echocardiographic window than M-mode echocardiography. The aim of this study was to assess echocardiographic parameters associated with pulmonary arterial hypertension, defined by increasing right ventricular systolic pressure(RVSP), calculated from trans-tricuspid gradient in patients with chronic hypoxic lungs. Method : We examined 19 patients with chronic hypoxic lung disease, suspected pulmonary hypertension under the clinical guidelines by two dimensional echocardiography via the left parasternal and subcostal approach in a supine position. Doppler echocardiography measured RVSP from tricuspid regurgitant velocity in continuous wave with 2.5MHz transducer and acceleration time(AT) on right ventricular outflow tract in pulsed wave for the estimation of pulmonary arterial pressure. Results : On echocardiography, moderate to severe degree of pulmonary arterial hypertension was defined as RVSP more than 40mmHg, presenting tricuspid regurgitation. Increased right ventricular endsystolic diameter and shortened AT were noted in the increased RVSP group. Increased RVSP was correlated negatively with the shortening of AT. Other clinical data, including pulmonary functional parameters, arterial blood gas analysis and M mode echocardiographic parameters were not changed significantly with the increased RVSP. Conclusion : These findings suggest that shortened AT on pulsed doppler can be useful when quantifying pulmonary arterial pressure with increased RVSP in patients with chronic lung disease with hypoxemia. Doppler echocardiography in pulmonary hypertension of chronic hypoxic lungs is an useful option, based on noninvasiveness under routine clinical practice.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1212-1218
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• 2005

Purpose : Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. Methods : Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. Results : Ten(67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. $O_2$ saturation and pH were lower and cardiothoracic(CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia(P<0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but $O_2$ saturation was lower in functional atresia group(P<0.05). 13 patients(87%) were managed with $PGE_1$. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period(mean 37 months). 3 patients(20%) died and none of patients without pulmonary atresia died. Conclusion : We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.

• Journal of Chest Surgery
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• v.32 no.3
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• pp.237-248
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• 1999

Background: The late results of repair of tetralogy of Fallot(TOF) are favorable in most patients. Some portion of the patients with tetralogy, however, require reoperation for residual lesions or result in late death. The outcome of patients after tetralogy repair performed during the past 13 years was studied, with an emphasis on postrepair survival and problems including reoperations. Material and Method: A retrospective review of clinical, echocardiographic and catheterization data was performed in 569 of 775 patients with TOF who underwent corrective repair between 1983 and 1995 at Sejong General Hospital, Buchon, Korea. Result: Of 28(4.9%) early deaths(defined as 30 days postrepair), 12 deaths(42.9%) were <1 year of age, with an operative mortality of 15.4%. The surviving 541 patients(age 2.8 months to 43.4 years, median 23 months) have been followed up from 1 month to 12.6 years(median 35 months) postoperatively. Most patients were in good functional class and had normal right ventricular(RV) function. Postrepair results were compared between the transatrial-transpulmonary approach and the conventional right ventriculotomy. The former technique provided a lesser incidence of significant pulmonary regurgitation(P<0.001) and alesser degree of RV dysfunction(P<0.05) compared with those in the latter. There were 10(1.8%) late deaths during the follow-up period and 6 of the deaths were directly related to reoperation or ventricular dysfunction. The 10-year actuarial survival rate was 96.7%. There were 44 reoperations(8.1%) in 39 patients(7.2%), with an operative mortality of 10.3%. The main indications or reoperation included residual ventricular septal defect(VSD) (n=6), pulmonary stenosis(PS) (n=11), VSD with PS(n=17), pulmonary regurgitation(n=7), and tricuspid regurgitation(n=2). The 5- and 10-year freedom from reoperation were 89.4% and 76.1%, respectively. Conclusion: Although the majority of patients with repaired TOF are clinically well, with a high rate of survival, approximately 7% of patients have residual lesions that require surgical therapy. Therefore, the timely and meticulous corrective repair is mandatory to avoid reoperation, and continued close surveillance is also needed for the early detection of residual problems.

• 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.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.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

• Tuberculosis and Respiratory Diseases
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• v.59 no.6
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• pp.644-650
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• 2005

Background : Bilateral pulmonary thromboendarterectomy(PTE) is recognized as the definitive treatment for chronic thromboembolic pulmonary hypertension (CTEPH). We investigated the symptomatic, hemodynamic and prognostic effects of PTE in comparison with medical treatment. Methods : Twenty-four patients diagnosed with CTEPH from 1995 to 2003 at the Asan Medical Center were divided into two groups: patients treated with PTE(PTE group, n=12) and those not treated with PTE(Med group, n=12). The serial changes in dyspnea, the tricuspid regurgitation maximal velocity (TRVmax) and survival of the PTE and Med groups were compared retrospectively. Results : In PTE group, during a follow-up period of 1 year, the New York Heart Association(NYHA) functional class significantly improved, while there was no significant improvement in the Med group. PTE significantly lowered the TRVmax from $4.23{\pm}0.54m/sec$ to $3.22{\pm}0.70m/sec$ over a follow up period of 2 years. (p=0.028) However, the TRVmax in the medically treated group did not show any significant improvement, changing from $3.98{\pm}0.68m/sec$ to $4.27{\pm}0.95m/sec$ during 1 year. The 5-year survival of the PTE group was 77.9% compared with 64.3% in the medically treated group. Conclusion : PTE provides substantial long-term improvement in dyspnea and the echocardiographic changes compared with medically treated patients.