• Journal of Chest Surgery
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• v.34 no.10
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• pp.787-791
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• 2001

Coronary artery aneurysm is a rare disorder. It is defined as abnormal dilatation of coronary artery with diameter exceeding 1.5 times the adjacent normal segments. The incidence of coronary aneurysm is 2.6% in Caucasians and 0.25% in Asians. Over half of the former were associated with atherosclerotic coronary artery disease. However, 70 percents of the latter were nonobstructive coronary artery aneurysms. Coronary artery fistula is a rare disorder. It has been identified in only 0.2% of routine cardiac angiographic studies conducted over a 10-year period. The clinical spectrums are various, asymtomatic, asymptomatic murmur, dyspnea on exertion, fatigue, and congestive heart failure. The right coronary artery (56%) and left coronary artery(36%) are mainly involved in the origin site of congenital coronary artery fistula. The draining site of fistula are right ventricle(39%), right atrium(33%), and pulmonary artery(20%) and so on. This 54 years-old woman had intermittent chest tightness and an abnormal mediastinal shadow on chest roentgenogram and chest C-T examination, which was diagnosed as a mediastinal mass such as teratoma. We performed the operation under left anterolateral thoracotomy for mass excision. However, we knew the mass had the pulsating arterial blood flow through a fine needle puncture of the mass and that it was attached to the left ventricle. We believed the excision of mass on beating heart would be very dangerous. Therefore, we closed the wound without excising the mass. After several days, we performed an echocardiography and coronary angiography, We knew it was cardiac tumor. Incidentally, the patient had a tortuous coronary fistula from the right coronary artery to pulmonary trunk. Using cardiopulmonary bypass with moderate systemic hypothermia, the mass was resected and the fistula was clipped with surgical clips. Pathology of the specimen was a giant coronary arterial aneurysm.

• 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.29 no.12
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• pp.1360-1365
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• 1996

From September 1992 to May 1996, 38 patients ranging in age from 23 to 78, were operated for aortic dissection at Asan medical center There were 21 men and 17 women. The underlying aortic pathology were acute aortic dissection in 23, chronic aortic dissection in 15. Eight patients had Martian syndrome. In 34 cases of DeBakey type I, II patients, femoral artery and vein and/or right atrial auricle were used as cannulation site. With deep hypothermic c rculatory arrest (esophageal temperature 12 $\pm$ 2.5$^{\circ}C$) and retrograde cerebral perfusion of cold oxygenated blood through SVC, we replaced the ascending aorta and the part of arch if necessary. The mean duration of the total circulatory arrest time was 25 $\pm$ 1.7 mintstuts. In 4 cases of DeBakey type III patients, we replaced descending thoracic aorta or thoracoabdomlnal aorta without shunt or bypass under normothermia with an average 30: 1.5 minutesaortic cross clamp time. One death(2.6%) occurred on the twenty-second postoperative day owing to asphyxia related to ulcer bleeding. Postoperative complications were myocardial infarction with transient left peroneal palsy in 1 case, transient lower extremity weakness in 1 case and prolonged ventilatory support in 1 case. Two patients required reoperation due to retrograde extended dissection and aortic insufuciency. There was no late death with an average 25 months follow-up period.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.430-438
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• 2002

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. We reviewed our recent surgical experiences on congenital heart defect (other than patent ductus arteriosus) in low birth weight babies. Material and Method: From September 1994 to February 2001, 31 consecutive infants weighing 2500 g or less underwent cardiac surgery with (OHS group n=12) or without cardiopulmonary bypass (CHS group n=19). A retrospective study was carried out to evaluate short-and intermediate-term outcome. Mean gestational age and age at operation were 36.9 weeks(range, 32.3-42weeks) and 32.1days (range, 0-87days) respectively. Mean body weight at birth and operation were 1972g (range, 1100-2500g) and 2105g (range, 1450-2500 g) respectively. There was no difference between the two groups in age and body weight. Defects included ventricular septal defect (VSD) (n=3), VSD with arch anomaly (n=2), total anomalous pulmonary venous return (n=2), transposition of the great arteries (TGA) (n=2), truncus arteriosus (n=2), and univentricular heart with cor triatriatum (n=1) in OHS group, and coarctation of aorta (n=7), tetralogy of Fallot (TOF) (n=3), TOF with pulmonary atresia (n=3), multiple muscular VSDs (n=1), double outlet right ventricle (n=1), pulmonary atresia with intact ventricular septum (n=2), tricuspid atresia (n=1), and TGA with multiple VSD (n=1) in CHS group. 13 patients (41.9%) were intubated pre-operatively. Result: There were 4 early deaths(＜30 days); 1 (8.3%) in OHS group and 3 (15.8%) in non-OHS group. All these early deaths were related to the pulmonary artery banding(PAB). There was no operative mortality in infants undergoing complete repair and palliative operations other than PAB. Delayed sternal closure was required in 3 patients. Prolonged postoperative mechanical ventilation (>7 days) was required in 7 patients(58.3%) in OHS and 7(38.8%) in CHS group. Late mortality occurred in 3 patients, two of which were non-cardiac. A patient in OHS group was documented to have neurologic sequelae. All the survivors except two are in NYHA class 1. Conclusion: Complete repair and palliative operations other than PAB can be performed in low birth weight infants with low operative mortality and an acceptable intermediate-term result. However, about a half of the patients required long-term postoperative mechanical ventilation.

• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.275-280
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• 2006

Background: Aortopulmonary window (APW) is a very rare congenital heart anomaly, often associated with other cardiac anomalies. It causes a significant systemic to pulmonary artery shunt, which requires early surgical correction. Accurate diagnosis and surgical correction will bring good outcomes. The purpose of this study was to describe our 20-year experience of aortopulmonary window. Material and Method: Between March 1985 and January 2005, 16 patients with APW underwent surgical repair. Mean age at operation was $157.8{\pm}245.3$ ($15.0{\sim}994.0$) days and mean weight was $4.8{\pm}2.5$ ($1.7{\sim}10.7$) kg. Patent ductus arteriosus (8), atrial septal defect (7), interruptedaortic arch (5), ventricular septal defect (4), patent foramen ovate (3), tricuspid valve regurgitation (3), mitral valve regurgitation (2), aortic valve regurgitation (1), coarctation of aorta (1), left superior vena cavae (1), and dextrocardia (1) were associated. Repair methods included 1) division of the APW with primary closure or patch closure of aorta and pulmonary artery primary closure or patch closure (11) and 2) intra-arterial patch closure (3). 3) Division of the window and descending aorta to APW anastomosis (2) in the patients with interrupted aortic arch or coarctation. Result: There was one death. The patient had 2.5 cm long severe tracheal stenosis from carina with tracheal bronchus supplying right upper lobe. The patient died at 5th post operative day due to massive tracheal bleeding. Patients with complex aortopulmonary window had longer intensive care unit and hospital stay and showed more morbidities and higher reoperation rates. 5 patients had reoperations due to left pulmonary artery stenosis (4), right pulmonary artery stenosis (2), and main pulmonary artery stenosis (1). The mean follow-up period was $6.8{\pm}5.6$ (57.0 days$\sim$16.7 years)years and all patients belonged to NYHA class 1. Conclusion: With early and prompt correction of APW, excellent surgical outcome can be expected. However, optimal surgical method needs to be established to decrease the rate of stenosis of pulmonary arteries.

• Journal of Chest Surgery
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• v.34 no.10
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• pp.754-762
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• 2001

Background: To evaluate the efficacy of arterial switch operation for transposition of great arteries, serial echocardiographic studies were performed in 8 patients who underwent the surgery between 1989 and 1998 at Dept. of Thoracic & Cardiovascular Surgery, Yeungnam University Hospital in Daegu City, Korea. Material and Method: Follow-up period ranged from 6 months to 11 years(average of 4.1 years). Body weight ranged from 2.6kg to 4.8kg, with average of 3.6kg. 5 of 8 patients were preoperatively diagnosed as TGA+VSD, and 3 as TGA+IVS. LV function was evaluated by the measurement of LV shortening fraction, LVSTI, and LVEF. RVSTI was also measured. Postoperative function of valve and growth of great vessels were analyzed by the measurement of PSPGV, valvular regurgitation, LA/AO ratio, root dimension of aorta and pulmonary artery, comparing with the age matched controls, respectively. Result: LVEF had an average of 65.0+9.03% which is tended to increase serially. LVAOPG had an average of 15.9mmHg. RVPAPG, 27.5mmHg. From the measurement of aortic root dimension of 6 patients at end-systole, aortic root growth was assumed to increase more than the mean value of normal growth. PA root dimension at end-systole showed a similar growth progress when compared with age matched normal controls. Postoperative pulmonic valve regurgitation was noted in 5 of total 8 patients, in which 1 patient who showed grade 2 and 4 showed below grade 1. AR, in 6 patients and all grade 1 Except 1 patient, all the valvular regurgitations were below grade 1, which was presumed to be clinically insignificant.

• Journal of Life Science
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• v.31 no.10
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• pp.913-921
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• 2021

Limb-girdle muscular dystrophy (LGMD) which is characterized by progressive muscle weakening of the hip and shoulder shows both dominant and recessive inheritances with many pathogenic genes including TTN. This study performed to identify genetic causes of a male patient with late onset (45 years old) autosomal recessive LGMD and atrial flutter. By application of the whole exome sequencing, we identified bi-allelic variants of TTN gene in the patient. One allele had a single missense variant of [c.24124G>T (p.V8042F)], while the other allele consisted of three missense variants of [c.29222G>C (p.R9741P) + c.67490A>G (p.H22497R) + c.75376C>T (p.R25126C)]. The p.V8042F allele was transmitted from his mother, while the other haplotype allele was putatively transmitted from his father. His two unaffected sons had only the p.R9741P. These variants have been not reported or rarely reported in the public human genome databases (1,000 Genome, gnomAD, and KRGDB). Most variants were located in the highly conserved immunoglobulin or fibronectin domains and were predicted to be pathogenic by the in silico analyses. The TTN giant protein plays a key role in muscle assembly, force transmission at the Z-line, and maintenance of resting tension in the I-band. In conclusion, we think that these bi-allelic compound heterozygous mutations may play a role as the genetic causes of the LGMD phenotype.

• The Korean Journal of Nuclear Medicine Technology
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• v.23 no.2
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• pp.38-42
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• 2019

Purpose Although computed tomography (CT) is used for coronary artery calcification, it is difficult to differentiate between high risk microcalcifications. Studies have shown that $^{18}F$-sodium fluoride ($^{18}F-NaF$) is very useful for the diagnosis of microcalcifications. In this study, we aimed to evaluate the usefulness of $^{18}F-NaF$ PET imaging in quantitative evaluation of calcification. Materials and Methods A total of 45 patients ($67.1{\pm}6.9years\;old$) were injected with 250 MBq of $^{18}F-NaF$ for 1 hour and images were acquired for 30 minutes. All patients underwent CT angiography (CTAngiography, CTA) before the PET scan. The SUVmax of calcification was measured and the background radioactivity of the left atrium was measured to determine Target to Background (TBR) and quantitatively analyzed. High risk group was classified through ROC curve (Receiver Operating Characteristic Curve). Results There were 226 coronary artery calcifications in the cohort and SUVmax was $1.15{\pm}0.39$. Of the 28 patients (62%), 58 were classified as high risk (TBR > 1.25). The remaining 168 were $TBR{\leq}1.25$. Conclusion $^{18}F-NaF$ PET images were available for quantitative assessment of microcalcifications and could be classified into high-risk groups. The combination of angiographic CT and $^{18}F-NaF$ PET may be a new method for early diagnosis of high-risk microcalcifications.

• Journal of Chest Surgery
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• v.41 no.3
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• pp.329-334
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• 2008

Background: Percutaneous cardiopulmonary support. (PCPS) has the potential to rescue patients in cardiogenic shock who might otherwise die. PCPS has been a therapeutic option in a variety of the clinical settings such as for patients with myocardial Infarction, high-risk coronary intervention and postcardiotomy cardiogenic shock, and the PCPS device is easy to install. We report our early experience with PCPS as a life saving procedure in cardiogenic shock patients due to acute myocardial infarction. Material and Method: From January 2005 to December 2006, eight patients in cardiogenic shock with acute myocardial infarction underwent PCPS using the CAPIOX emergency bypass system($EBS^{(R)}$, Terumo, Tokyo, Japan). Uptake cannulae were inserted deep into the femoral vein up to the right atrium and return cannulae were inserted into the femoral artery with Seldinger techniques using 20 and 16-French cannulae, respectively. Simultaneously, autopriming was performed at the $EBS^{(R)}$ circuit. The $EBS^{(R)}$ flow rate was maintained between $2.5{\sim}3.0L/min/m^2$ and anticoagulation was performed using intravenous heparin with an ACT level above 200 seconds. Result: The mean age of patients was $61.1{\pm}14.2$ years (range, 39 to 77 years). Three patients were under control of the $EBS^{(R)}$ before percutaneous coronary intervention (PCI), three patients were under control of the $EBS^{(R)}$ during PCI, one patient was under control of the $EBS^{(R)}$ after PCI, and one patient was under control of the $EBS^{(R)}$ after coronary bypass surgery. The mean support time was $47.5{\pm}27.9$ hours (range, 8 to 76 hours). Five patients (62.5%) could be weaned from the $EBS^{(R)}$ after $53.6{\pm}27.2$ hours. (range, 12 to 68 hours) of support. All of the patients who could successfully be weaned from support were discharged from the hospital. There were three complications: one case of gastrointestinal bleeding and two cases of acute renal failure. Two of the three mortality cases were under cardiac arrest before $EBS^{(R)}$ support, and one patient had an intractable ventricular arrhythmia during the support. All of the discharged patients are still surviving at $16.8{\pm}3.1$ months (range, 12 to 20 months) of follow-up. Conclusion: The use of $EBS^{(R)}$ for cardiogenic shock caused by an acute myocardial infarction could rescue patients who might otherwise have died. Successfully recovered patients after $EBS^{(R)}$ treatment have survived without severe complications. More experience and additional clinical investigations are necessary to elucidate the proper installation timing and management protocol of the $EBS^{(R)}$ in the future.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.463-471
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• 2003

The long-term results of combined mitral valve repair and aortic valve replacement (AVR) have not been well evaluated. This study was performed to investigate the early and long-term results of mitral valve repair with AVR. Material and Method: We retrospectively reviewed 45 patients who underwent mitral valve repair and AVR between September 1990 and April 2002. The average age was 47 years: 28 were men and 17 women. Twelve patients had atrial fibrillation and three had a previous cardiac operation. The mitral valve disease consisted of pure insufficiency (MR) in 34 patients, mitral stenosis (MS) in 3, and mixed lesion in 8. Mitral valve disease was due to rheumatic origin in 24 patients, degenerative in 11, annular dilatation in 8, and ischemia or endocarditis in 2. The functional anatomy of mitral valve was annular dilatation in 31 patients, chordal elongation in 19, leaflet thickening in 19, commissural fusion in 13, chordal fusion in 10, chordal rupture in 6, and so on. Aortic prostheses used included mechanical valve in 32 patients, tissue valve in 12, and pulmonary autograft in one. The techniques of mitral valve repair included annuloplasty in 32 patients and various valvuloplasty of 54 techniques in 29 patients. Total cardiopulmonary bypass and aortic cross clamp time were 204$\pm$62 minute and 153$\pm$57 minutes, respectively. Result: Early death was in one patient due to low output syndrome (2.2%). After follow up of 57$\pm$37 months, late death was in one patient and the actuarial survival at 10 years was 96$\pm$4%. Recurrent MR developed grade II or III in 11 patients and moderate MS in 3. Three patients required reoperation for valve-related complications. The actuarial freedom from recurrent MR, MS, and reoperation were 64$\pm$11%, 86$\pm$8%, and 89$\pm$7% respectively. Conclusion: Combined mitral valve repair with AVR offers good early and long-term survival, and adequate techniques and selection of indication of mitral valve repair, especially in rheumatic disease, are prerequisites for better long-term results.