• Journal of Chest Surgery
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• v.30 no.10
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• pp.979-985
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• 1997

Between October 1991 and May 1995, 256 "New Duromedics Valve"(Edward TEKNA Bileaflet Valve) were implanted in 208 adult patients(171 mitral, 82 aortic and 3 tricuspid) with age ranging from 18 years to 70 years(mean 48.2$\pm$ 11.6 years). Postoperative complication rates were 12.2%, but there was none valve related one. Overall early mortality rate were 1.4%(1.6% for MVR, 2.1% for DVR, and none for AVR or TVR) respectively. Follow-up was 99% completed ranging in duration from 2 months to 46 months. There were 6 valve-related late complications(2.9%) with 2 patients with upper gastrointestinal bleeding, 2 with cerebral thxomtioembolism, 1 with valve thrombosis and 1 with valve endocarditis. Freedom from these valve-related major complications were 89.9% at 40 months. There were 5 late deaths(2.4%). one of these late deaths was considered valve-related. Overall actuarial survival rates at 40 months were 95.5%, 96.8% for mitral, 97.1% for aortic, 100% for tricuspid, and 92.0% for double valve replacement respectively. Preoperative New York Heart Association functional class were 2.9, and 1.3 in post-operative state. We have been trying to keep the international normalized ratio(INR) with range of 2.5 to 3.0. The INR of 4 patients of 5 with anticoagulant ralated complications was beyond the range. To reduce the rate of anticoagulant related complications, we felt very strongly that the INR should be kept between 2.5 and 3.0. In our cases, there was no structural failure or significant hemolysis in the absence of periprosthetic leak. This experience encourages us to continue using the "New Duromedics Valve".omedics Valve".uot;.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Journal of Chest Surgery
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• v.38 no.5 s.250
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• pp.335-348
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• 2005

Background: This retrospective review examines the preoperative condition, postoperative course, mortality and cause of death for the patients who underwent modified Blalock-Taussig shunt for complex congenital heart defects in early infancy. Material and Method: Fifty eight patients underwent modified Blalock-Taussig shunts from January 2000 to November 2003. The mean age at operation was $23.1\pm16.2$ days ($5\~81\;days$), and the mean body weight was $3.4\pm0.7\;kg\;(2.1\~4.3\;kg)$. Indications for surgery were pulmonary atresia with ventricular septal defect in 12 cases, pulmonary atresia with intact ventricular septum in 17, single ventricle (SV) in 18, and hypoplastic left heart syndrome (HLHS) in 11. Total anomalous pulmonary venous return (TAPVR) was associated with SV in 4 cases. Result: There were 11 ($19.0\%$) early, and 5 ($10.6\%$) late deaths. Causes of early death included low cardiac output in 9, arrhythmia in 1, and multiorgan failure in 1. Late deaths resulted from pneumonia in 2, hypoxia in 1, and sepsis in 1. Risk factors influencing mortality were preoperative pulmonary hypertension, metabolic acidosis, use of cardiopulmonary bypass, HLHS and TAPVR. Twenty four patients ($41.4\%$) had hemodynamic instability during the 48 postoperative-hours. Six patients underwent shunt revision for occlusion, and 1 shunt division for pulmonary overflow. Conclusion: Modified Blalock-Taussig shunt for complex congenital heart defects in early infancy had satisfactory results except in high risk groups. Many patients had early postoperative hemodynamic instability, which means that continuous close observation and management are mandatory in this period. Aggressive management may appear warranted based on understanding of hemodynamic changes for high risk groups.

• Journal of Chest Surgery
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• v.36 no.5
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• pp.335-342
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• 2003

Background: The aortic arch replacement in an acute aortic dissection is technically demanding procedure that has a lot of postoperative morbidity and high mortality The authors have applied several techniques of aortic arch replacement to overcome the risks of the procedure. Therefore we analysed the results of these techniques. Material and Method: From March of 1996 to July of 2002, we performed 31 cases of the aortic arch replacement in the Stanford type A acute aortic dissection. There were 12 male and 19 female patient's with 59.6$\pm$9.4 years of mean age. Among them 18 cases were treated with the hemiarch replacement and 13 cases with the total arch replacement. We approached the aortic arch through median sternotomy in all but 3 cases of Clamshell incision and applied the deep hypothermic circulatory arrest with retrograde cerebral perfusion. The associated procedures were 2 Bentall's procedures, an axillobifemoral bypass, a femorofemoral bypass and a carotid artery bypass. Result: The postoperative morbidities were 8 acute renal failures, 3 CNS complications, 2 low cardiac output syndromes, 2 malpefusion syndromes, and 2 deep wound infections. There were 4 cases of early hospital mortality which were from an acute renal failure a postoperative bleeding, a low cardiac output syndrome, and a reperfusion syndrome. There were 3 cases of late hospital mortality which were from an acute renal failure, and 2 multiorgan failures. So the total mortality rate was 22.5%. There were 4 cases of late mortality after the discharge, which were form 2 cases of distal anastomotic rupture and 2 cases of intracranial hemorrhage. Conclusion: The hemiarch replacement has relatively shorter operative time and lower hospital mortality but higher late mortality than the total arch replacement. The total arch replacement needs more technically demanding procedure.

• Journal of Chest Surgery
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• v.35 no.3
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• pp.171-176
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• 2002

Background: Blood-foreign interaction cause activation of coagulation and inflammatory process that may lead to multiorgan dysfunction and determine the surgical outcomes. Of the methods for assessing the biocompatibility, the platelet adhesion study is considered as the most valuable evaluation step in blood-foreign interaction. As the most studies have used in-vitro or ex-vivo conditions, we have developed a technique of quantification for platelet adhesion on the blood contact surface by using in-vivo injection of radioactive platelets. Material and Method: A coupled bypass circuit was designed to connect the proximal and descending thoracic aorta in 6 piglets(20∼25 Kg). One side of the circuit tube was consisted of a heparin coated PVC tube(10mm in ID, n=6, Experimental group), and the other, a non-heparin coated PVC tube(10mm in ID, n=6, Control group). After cannulation, the blood was circulated through the circuit for 2 hours. Platelet concentrate was prepared from homologous pig blood 24 hours before the experiment. The platelet concentrate was incubated with Tc-99m-HMPAO for 30 min and then centrifuged for 10 min. The supernatant was discarded and the radio-labeling efficacy was measured. The radio-labeled platelet concentrate was mixed with the autologous plasma to make the volume 5 ml, and the mixture was injected intravenously into the experimental animal. After 2 hour circulation, 5 pieces of the specimen(10mm in length each) were obtained from each PVC tube. The radioisotopes were counted with a gamma counter(Cobra ll, Packard, USA), and the ratio of radioisotope count was compared between the control and experimental group. Result: The radioisotope count number was 537.3221.1 Ci/min in the control group and 311.1 184.5 Ci/min in the experimental group(p=0.0104). The ratio between the groups was 1 to 0.58 (p=0.004). Conclusion: In vivo quantification using technetium-99m-HMPAO labeled platelets is simple and reproducible in evaluating platelet adhesion on a foreign surface. We suggest this technique to be a useful tool for blood compatibility test.

• Tuberculosis and Respiratory Diseases
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• v.65 no.4
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• pp.292-300
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• 2008

Background: Respiratory failure is a common condition that requires intensive care, and has a high mortality rate despite the recent improvements in respiratory care. Previous reports of patients with respiratory failure focused on the specific disease or included a large proportion of surgical patients. This study evaluated the clinical characteristics, outcomes and prognostic factors of adult patients receiving mechanical ventilation in a medical intensive care unit. Methods: Retrospective chart review was performed on 479 adult patients, who received mechanical ventilation for more than 48 hours in the medical ICU of one tertiary referral hospital. Results: The mean age of the patients was $60.3{\pm}15.6$ years and 34.0% were female. The initial mean APACHE III score was $72.3{\pm}25$. The cause of MV included acute respiratory failure (71.8%), acute exacerbation of chronic pulmonary disease (20.9%), coma (5.6%), and neuromuscular disorders (1.7%). Pressure controlled ventilation was used as the initial ventilator mode in 67.8% of patients, and pressure support ventilation was used as the initial weaning mode in 83.6% of the patients. The overall mortality rate in the ICU and hospital was 49.3% and 55.4%, respectively. The main cause of death in hospital was septic shock (32.5%), respiratory failure (11.7%), and multiorgan failure (10.2%). Males, an APACHE III score >70, the cause of respiratory failure (interstitial lung disease, coma, aspiration, pneumonia, sepsis and hemoptysis), the total ventilation time, and length of stay in hospital were independently associated with mortality. Conclusion: The cause of respiratory failure, severity of the patients, and gender appears to be significantly associated with the outcome of mechanical ventilatory support in patients with respiratory failure.

• Tuberculosis and Respiratory Diseases
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• v.62 no.2
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• pp.113-118
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• 2007

Background: Paraquat is known to induce oxidant injury that results in multiorgan failure and lung fibrosis. Iron has been considered to play a key role in paraquat-induced oxidant lung injury. This study examined the effect of deferoxamine, an iron-chelating agent, in the treatment of paraquat poisoning. Methods: From September, 2001 to April, 2005, 28 patients with paraquat poisoning who were admitted at a medical intensive care unit of a University-affiliated hospital, were enrolled in this study. Sixteen patients were treated according to the paraquat poisoning treatment guidelines and 12 received an intravenous infusion of deferoxamine in addition to the treatment guidelines. Results: There were no differences between the two groups in terms of age, gender, severity of paraquat poisoning, and the time elapsed from ingestion to presentation at hospital. There was no difference in overall mortality between the two groups but the incidence of respiratory failure in the deferoxamine group was higher than in the conventional group(4/7 versus 0/9, p=0.019). Conclusion: Deferoxamine seems to have no clinical benefit compared with the conventional treatment.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1218-1222
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• 1996

The reported incidence of postcardiotomy cardiogenic shock not responding to conventional therapy is still 0.1 to 0.8%. For this group of patients, more aggressive form of circulatory support must be employed. Centrifugal pumps are a ventricular assist device most commonly used on this purpose, due to low cost and easy availability. Currently, however, clinical experience of centrifugal pumps as a ventricular assist device is rarely reported in Korea. From January 1992 to January 1996, 2986 patients underwent cardiac operations on cardiopulmonary bypass at Seoul National University Hospital. Refractory postcardiotomy cardiac failure requring ventricular support with a Biomedicus centrifugal pump developed in ten of these patients. There were eight men and two women, ranged in age from nine years to 77 years with a mean of 50$\pm$20 years. The primary surgical procedures consisted of isolated coronary revascularization in four patients, combined coronary revascularization and aortic valve replacement in two, aortic dissection repair in two, pulmonary embolectomy in one, and heart transplantation in one. Of the ten patients, five had left ventricular assistance, one had right ventricular assistance, and four had biventricular assistance. Duration of ventricular assistance ranged from 24 to 175 hours, with a mean of 76$\pm$51 hours. Seven patients were weaned from ventricular assistance, and four of them discharged. The causes of death for nonsurvivors were progressive cardiac failure in two patients and multiorgan failure, intractable ventricular fibrillation, irreversible brain injury, and mechanical problem, respectively, in the other four. Survival was not predicted by time on cardiopulmonary bypass, aortic cross-clamp time, or duration of ventricular support. Major complications included bleeding(7), renal failure(6), infection(3) and neurologic complication(2). These results indicate that a centrifugal pump can provide reasonably satisfactory short-term circulatory support.