• Journal of Chest Surgery
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• v.42 no.1
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• pp.87-91
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• 2009

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.23 no.3
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• pp.263-269
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• 2001

Median cleft of the lower lip and/or mandible is a rare congenital anomaly, first mentioned by Couronne in 1819. Monroe(1966), Fujino(1970), Ranta(1984) and Oostrom(1996) conducted comprehensive reviews and list cases in literature. Median cleft varies greatly, from a simple vermilion notch to a complete cleft of the lip involving the tongue, the chin, the mandible, the supporting structures of the median of the neck, and the manubrium sterni. The associated anomalies include ankyloglossia, cleft tongue, neck contraction, heart lesion, absence of hyoid bone, and so on. The etiology of median cleft is unknown. Various possibilities, such as failure of mesodermal penetration into the midline, failure of fusion of mandibular processes, external factors apart from the embryogenic pattern such as pressure, position in utero, circulatory failure caused placental adhesion, diseases in pregnancy, and so on, have been discussed. A 8-year-old girl was referred to the Dept. of Oral & Maxillofacial Surgery, Kyungpook National University Hospital and had been aware of the fact that at birth "she had something wrong with her mouth." Shortly after birth she had been examined by a plastic surgeon and at that time surgical procedure had been performed to release the tongue from the lower jaw and lip at local hospital. On admission, she had a slight notching of lower lip and two fibrous frenum ran from the lip along the ventral surface of the tongue, diastema between her mandibular central incisors, and slightly constricted bifid mandible associated independent movement of the two halves of mandible. The patient had autogenous iliac bone graft to reconstruct the mandibular midline defect. The postoperative result was uneventful. In future, the correction of the soft tissue deformities such as notching of the lower lip and partial ankyloglossia will be required for the esthetic and functional improvement.

• Journal of Chest Surgery
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• v.21 no.2
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• pp.211-222
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• 1988

Tetralogy of Fallot is a cyanotic congenital heart disease characterized by large ventricular septal defect[VSD] and stenosis of right ventricular outflow tract[RVOT] and the degree of RVOT stenosis and the state of pulmonary arteries are the major determinant of prognosis of this anomaly after operation. The sum of blood flow through RVOT and collateral flow from systemic arteries determine the total pulmonary blood flow and it is drained to left atrium and left ventricle. Therefore the degree of development of left ventricle not only reflects pulmonary blood flow and the status of peripheral pulmonary arteries but also affects postoperative prognosis as a systemic ventricle. In this article, left ventricular volume and its influence on postoperative cardiac function in tetralogy of Fallot were studied in 34 patients operated on at Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital in 1985. Mean age of the patients was 5 1/12*3 9/12 years[range 9/12 - 14 8/12 years], mean body surface area[BSA] 0.65*0.20m2[range 0.38 - 1.22m2], mean body weight 15.6k6.48kg[range 7.0 - 36kg]and mean hematocrit 50.6*9.77%[range 32.0 - 73.5%]. Left ventricular end-diastolic volume[LVEDV] of them were from 11.2 to 113cc and there was a good linear correlation between BSA[m2, X]and LVEDV[cc, Y][Y= - 20.0+923x, r= 0.84, p < 0.005]. Mean LVEDV/m2 was [57.6 * 18.3 cc / m2[range 28.7 - 95.8 cc / m2] and there was a significant reduction of volume compared with normal value. As body surface increases, there was a increasing tendency in LVEDV/m2 but there was no statistical significance. Mean total amount of postoperatively infused dopamine in these 33 patients[except one who expired postoperatively] was 65.6*74.5mg / kg and it was 40.6*44.0mg / kg in routine RVOT widening group [Group I] and 205*49.3mg / kg in transannular RVOT widening group[Group II]. There was a statistically significant difference between two groups. In group I patients there was a good linear inverse correlation between dopamine total amount[mg / kg, Y] and LV volume[cc / m2, X] [Y = 150 - 1.89 X, r = - 0. 77, p < 0.005]. But there were no correlations between dopamine total amount and Hct, cardiopulmonary bypass time and aorta cross clamp time. In conclusion, the patient with small preoperative left ventricular volume required more amount of dopamine as an inotropic agent for the maintenance of a cardiac function in postoperative period. But this is a result of immediate postoperative period and does not reflect the long term effect of left ventricular volume in tetralogy of Fallot. There must be more study for the evaluation of its long term effect.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.

• 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.42 no.3
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• pp.299-304
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• 2009

Background: Although the results of the surgical management for complete atrioventricular septal defect (c-AVSD) have improved, the optimal surgical strategy is still controversial. The aims of this study are to evaluate the outcome of c-AVSD repair and to define the risk factors related to reoperation. Material and Method: We retrospectively reviewed the medical records of 35 patients (8 males and 27 females) who underwent the total correction of c-AVSD from August 1996 to March 2008. The median age at repair was 5.2 months (range: 3 days$\sim$82 months). Sixteen patients (45.7%) were associated with Down syndrome. Prior palliative operations were performed in 4 patients. The one-patch techniques were performed in 3 patients, and the two-patch techniques were done in 32 patients. Result: There was 1 early death (2.9%). The median follow-up period was 68 months (range: $2\sim134$ months) for 34 survivors. There was no late death. Reoperations were performed in 5 patients (14.3%) for severe left atrioventricular valvular regurgitation (AVVR). Nine patients (25.7%) showed left an AVVR of more than grade III. Associated major cardiac anomalies and the use of Gore-Tex patch for ventricular septal closure were the risk factors for postoperative left atrioventricular valve failure and reoperation. Conclusion: In this study, we found that surgical repair of c-AVSD was safe and effective. However, the high reoperation rate after repair remains a problem to be solved.

• Journal of Chest Surgery
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• v.32 no.4
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• pp.341-346
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• 1999

Background: Thirty children ranging from 3 to 15 years of age underwent cardiac valve replacement at Dongsan Medical Center from 1982 to 1997. Material and Method: There were 16 boys and 14 girls. The mean age was 12.1. The underlying pathological cause for valve replacement was congenital heart disease in 17 children and acquired heart disease in 13. The valve replaced was mitral in 15 children, aortic in 11, tricuspid in 3, and combined aortic and mitral in 1. Twenty-one mechanical and 10 tissue valves were placed: primary mechanical valve have been utilized since 1985. Eight of ten patients with tissue valves have had successful second valve replacements 4 to 11 years after the initial operation. Result: The operative mortality was 6.7%, but mortality was higher among patients less than 5 years of age and patients who had previous cardiac operations. Of the 28 operative survivors, 4 patients were lost to follow-up: the remaining patients were observed for a total of 2091 patient/months(mean 74.7 months, maximum 187 months). There was one late death from dilated cardiomyopathy after mitral valve replacement in 7 year-old patient with atrioventricular septal defect. After the operation, all patients with mechanical valves were placed on a strict anticoagulant regimen with Coumadin. The actuarial survival rate was 96% at the end of the follow-up. No instance of thromboembolism or major bleeding were observed in the survivors. Conclusion: These results indicate that valve replacement can be performed with low mortality in children, and with satisfactory long-term survival.

• 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.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.30 no.2
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• pp.152-157
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• 1997

From August 1986 until June 1995, single aortic valve replacement was performed in 65 patients at the Chonnam National University Hospital. worthy-eight were male and 17 were female patients, ranging from 19 to 68 years of age(median : 43 years). The causes of the valve lesions were rheumatic in 29 patients (44.6%), bicuspid aortic valve in 6 patients (6.2%), endocarditis in 6 patients(6.2%), unknown in others. Concomitant surgical procedures were performed in 10 patients : repair of congenital defect in 5, pericardiectomy in 1, coronary artery bypass grafting in 1, noncoronary sinus plication in 1, Valsalva sinus aneurysmectomy in 1, subaortic membrane resection in 1 Used valves were 51. Jude-Medical valve in 42, Duromedics valve in 22, Bjork-Shiley valve in 2, Carpentier-Edward valve in 1. There were 3 hospital deaths (4.6%), and 2 late deaths (3.2%). Follow-up was 95.2% complete. The 10-year acturlal survival rate was 85.3%. Postoperative complications were low cardiac utput in 8, arrythmia in 5, valve related hemolysis in 1, cerebral infarction in 1, and gastrointestinal bleeding in 2. Reoperation was performed in 4 for surgical bleeding, in 3 for paravalvular leak. The mean improvement in New York Heart Association functional class is from 2.79 $\pm$ 0.66 preoperatively to 1.25 $\pm$ 0.49 postoperatively(p < 0.001) The change of cardiothoracic ratio from preoperative to postoperative is 0.57 $\pm$ 0.06 to 0.54 $\pm$ 0.05 (p < 0.05). The left ventricular ejection fraction change is not significant perioperatively. There are no mechanical failures. This early and intermediate-term follow-up suggests that in adults in whom valve repair is not possible, the mechanical valve is a reliable and durable prosthesis with good hemodynamic function and a low rate of thromboembolic event.