• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.154-156
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• 2006

Inferior sinus venosus ASD (atrial septal defect) is a rare congenital cardiac deformity, that occurs on between the inferior vena cava and right atrium. Diagnosis of inferior sinus venosus ASD is difficult because of its infero-posterior location of the fossa ovalis. Therefor, exact anatomical diagnosis by preoperative and intraoperative transesophageal echocardiography is necessary at preoperation and during the operation. We present a case of residual ASD, which was diagnosed secundum ASD and repaired when the patient was 10 years old. Residual ASD was diagnosed by cardiac echocardiography in preparation of otorhinolaryngology operation. Therefore, reoperation of residual ASD was done when the patient was 24 years old. The patient had secundum ASD and inferior sinus venosus ASD, but in the prior operation, inferior sinus venosus ASD wasn't found and only secundum ASD was repaired. In reoperation, inferior sinus venosus ASD was reveled and patch closure was done.

• Journal of Chest Surgery
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• v.36 no.12
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• pp.961-969
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• 2003

The purpose of this study was to evaluate the fate of left atrioventricular valve regurgitation(LAVVR) following repair of complete atrioventricular septal defects (AVSDs). Material and Method: Between July 1984 and March 2002, repair of complete AV defects were performed in 77 patients. Mean age at surgery was 30.23$\pm$69.11 months (range 1 to 456). Echocardiograms of all survivors after isolated AVSDs correction were reviewed. LAVVR were evaluated with color doppler echocardiography in 64 survival periodically. On each study, LAVVR severity was graded on a 1 to 4 scale, based upon the size of the regurgitated jet. Result: Mild deterioration of LAVV function was fairly common. LAVVR severity increased by >1 grade in 19 patients (30.2%) during the course of the study. However, the deterioration in LAVVR function occurred primarily between 12 and 24 months postoperatively. After the initial 24 postoperative months, LAVVR worsened on only 8 occasions and in each instance worsened by only 1 grade. Deterioration more than 3+ LAVVR occurred in only 3 patients. And deterioration to 4+ LAVVR was not observed after the initial 24 postoperative months but one. Survival curve analysis predicted a 88.2% of ten-year freedom rate from development of 4+ LAVVR after initial operation of complete AVSDs. Conclusion: Postoperative LAVVR remains fairly stable following AVSDs repair, Serious deterioration is rare after 24 postoperative months, especially after the initial 48 postoperative months. But serial follow-up study with echocariogram was need till 24 postoperative months after repair of complete AVSDs.

• Journal of Chest Surgery
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• v.38 no.3 s.248
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• pp.181-190
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• 2005

The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. Material and Method: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1$\~$44) and 86 patients ($68.3\%$) were male. We classified and studied this disease by the type of VSD. Result: The locations of VSD were subaortic in 79 ($62.7\%$), subpulmonary in 17 ($13.5\%$), doubly committed in 16 ($12.7\%$) and noncommitted in 14 ($11.1\%$). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0$\pm$45.1 months. The methods of total correction were intraventricular baffling in 37 ($29.4\%$), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 ($38.9\%$), intraventricular baffling with Rastelli procedure in 15 ($11.9\%$), arterial switch operation in 8 ($6.3\%$) and REV procedure in 4 ($3.2\%$), etc. Hospital mortality rate was $10.3\%$ (13 patients) and 25 reoperations were performed in 24 patients ($19.0\%$). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9$\pm$70.7 months. The percent survival and survival for freedom from reoperation at 15 years were $82.5\%$ and $66.7\%$, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. Conclusion: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.554-560
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• 2003

Purpose : To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. Methods : 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. Results : The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. Conclusion : Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.194-198
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• 2009

Purpose : Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC. Methods : Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included. Results : Mean age at diagnosis was $28.1{\pm}33.4$ days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up. Conclusion : In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.

• Journal of Chest Surgery
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• v.29 no.9
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• pp.983-988
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• 1996

Aneurysm of the sinus of Valsalva is fairly rare. Between 1987 and 1994, we operated on a total of 12 cases of aneurysm of the sinus of Valsalva at the Inje University Seoul Paik Hospital. This represents 0.7% of all cardiac operation under cardiopulmonary bypass. There were eight male and four female patients aged from 8 to 38 years(mean, 19.2 years). 8 of 12 patients had ruptured aneurysms. The origin of aneurysm of the sinus of Valsalva was the right coronary sinus in 10(83. 3%), and the noncoronary sinus in 2(16.6%). In ruptured aneurysm, the origin was the right oronary sinus in 6, and the noncoronary sinus in 2. The aneurysms originating from the right coronary sinus ruptured into the right ventricle in 5, and into both the right atrium and right ventricle in 1. The aneurysms originating from the noncoronary sinus ruptured into the right ventricle in 1, and into the right atrium in 1. Associated congenital cardiac defects included ventricular septal defect in 10(83.3%) patients 39 cases of these were associated with the aneurysms of the right coronary sinus), aortic regurgi- tation in 3 (all of these had an additional ventricular septal defect), mitral regurgitation in 1, and double chambered right ventricle in 1. No hospital deaths occurred, although one late death occurred as a result of endocarditis 15 months after the first operation. The mean follow-up period was 29 months, range from 4 to 60 months. Eleven patients except one late death were in New York Heart Association class 1. Due to the low mortality risk o( an operation for aneur sm of the sinus of Valsalva, a ruptured aneurysm of the sinus of Valsalva should be corrected surgically when the diagnosis is mane, and unruptured aneurysm of the sinus of Valsava with complication should also be operated. In most cases the aorta was opened to examine the morphology of the aneurysm and the aortic cusps, and an associated aortic valve defect should be corrected simultaneously.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.727-734
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• 2004

The Lecompte procedure for transposition of the great arteries has an advantage because it obviates the need for an extracardiac conduit for the reconstruction of the pulmonary outflow tract. We evaluated the effectiveness and the application of the Lecompte procedure. Material and Method: A retrospective review was conducted of the records of 46 patients who underwent the Lecompte procedure during the past 15 years. Mean age at operation was 29.2$\pm$20.3 (range: 3∼83) months. The diagnoses involved anomalies of the ventriculoarterial connection with ventricular septal defect and pulmonary outflow tract obstruction, such as transposition of the great arteries, double-outlet right ventricle, and double-outlet left ventricle. Result: Early mortality was 4.4% (2 of 46 patients) and late mortality was 6.8% (3 of 44). The mean follow-up was 11.2$\pm$6.9 years. Eighteen patients (43.9% of survivors, n=41) had pulmonary stenosis (pressure gradient above 30 mmHg), the main reason for which was a calcified monocusp valve (n=15, 83.3%). Seventeen of 46 patients (37.0%) underwent reoperation: 15 for pulmonary stenosis, 5 for residual ventricular septal defect, 4 for left ventricular outflow tract obstruction, 3 for pulmonary insufficiency, and 4 for other causes. The cumulative survival rates were 91.3$\pm$4.2%, and 87.0$\pm$5.8% at 10 and 15 years, respectively. The actuarial probabilities of freedom from reoperation for pulmonary stenosis were 90.6$\pm$4.5%, 73.9$\pm$7.3%, and 54.0$\pm$10.4% at 5, 10, and 15 years, respectively. Conclusion: The Lecompte procedure is an effective treatment modality. Repair in early age is possible with acceptable morbidity and mortality, but recurrent right ventricular outflow tract obstruction caused by degeneration of the monocusp valve is a problem that needs resolution.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.510-517
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• 1999

Background: Total anomalous pulmonary venous connection (TAPVC) is still one of the more challenging congenital heart defects in newborns and young infants. The purpose of the study is to evaluate the early and midterm results of the surgical corrections for patients in early infancy with isolated TAPVC. Material and Method: Hospital records of 15 consecutive patients in early infancy (January 1993 to August 1998) were retrospectively reviewed. There were 8 boys and 7 girls whose ages ranged from 4 days to 3.5 months (median age 22 days). Their body weight ranged from 1.75 kg to 4.9 kg (mean 3.54 kg). The abnormal anatomical connections were supracardiac in 11, cardiac in 3, and infracardiac in 1. In 6 patients (40%), the pulmonary venous drainage was obstructive. Total circulatory arrest was used in 13 patients. Anastomosis between the common pulmonary vein and the left atrium was performed with a continuous suture technique using a fine nonabsorbable polypropylene suture through a lateral approach behind the right atrium. Result: There was one hospital death (6.5%) caused by a sepsis 17 days after the operation in a neonate who had supracardiac drainage and was dependent on a ventilator preoperatively. There were 2 late deaths. One died sudde`nly of an unknown cause at home 2.5 years after the operation and the other died of a recurrent pulmonary hypertension 3 months after the reoperation due to pulmonary venous obstruction (PVO). Two patients required reoperations because of PVO 5 months and 10 months respectively after the initial operation. Of these patients, one patient is alive at the present time with persistent pulmonary hypertension. All survivors without postoperative PVO (78.6%) were in NYHA functional class I at mean follow-up of 25.8 months (0.5∼67 months). Conclusion: Surgical correction of TAPVC in early infancy can be performed at low risk. However, there were 2 postoperative PVOs (14.3%) which had bad results. The survivors without postoperative PVO had excellent functional status.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.498-504
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• 2002

Purpose : The purpose of this study was to evaluate the perioperative myocardial damage in pediatric congenital heart disease with the cardiac specific protein of cardiac troponin I(cTpn-I). Methods : All 25 pediatric patients who were diagnosed with tetralogy of Fallot or double outlet right ventricle were classified as group A(acyanotic, $SaO_2$ >90%), group B(mildly cyanotic, $SaO_2$ >80-90%) and group C(moderately cyanotic, $SaO_2$ <80%). The control group D was consisted of 10 patients with ventricular septal defects who were operated in the same period. We measured preoperative hemoglobin, preoperative and postoperative(24 and 72 hour) arterial oxygen saturation, cTpn-I and creatine kinase(CK-MB). Results : Total 25 patients were subdivided into 6 of group A, 12 of group B and 7 of group C. The concentrations of preoperative cTpn-I were $0.23{\pm}0.12ng/mL$ in group A, $0.25{\pm}00.12 ng/mL$ in group B, $0.26{\pm}0.13ng/mL$ in group C. And the concentrations of cTpn-I in postoperative 24 hour were $10.04{\pm}5.28ng/mL$ in group A, $12.50{\pm}6.86ng/mL$ in group B, $12.55{\pm}9.90ng/mL$ in group C. Which revealed cTpn-I in group C was higher than that of the another less cyanotic groups. In addition, the concentration of cTpn-I of group C in postoperative 72 hour was higher than any other groups. The concentration of cTpn-I in postoperative 72 hour was statistically correlated with that in postoperative 24 hour and preoperative arterial oxygen saturation(P=0.001). Conclusion : Preoperative chronic cyanosis can influence on the postoperative concentration of cTpn-I in pediatric cardiac patients, which means impairment on the postoperative myocardial recovery.