• Journal of Chest Surgery
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• v.32 no.5
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• pp.422-427
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• 1999

Background: Tachycardia induced heart failure model would be the model of choice for the dilated cardiomyopathy. This more closely resembles the clinical syndrome and does not require major surgical trauma, myocardial ischemia and pharmacological or toxic depression of cardiac function. When heart failure is progressive, application of new surgical procedures to the faling heart is highly risky. It has been shown that recovery trajectory from heart failure is a new method in decreasing animal mortality. The purpose is to establish the control datas for recovery trajectory in the canine heart failure model. Material and Method: 21 mongrel dogs were studied at 4 stages(baseline, at the heart failure, 4 and 8 weeks after recovery). Heart failure was induced during 4 weeks of continuous rapid pacing using a pacemaker. Eight weeks of trajectory of recovery period was allowed. Indices of left ventricular function and dimension were measured every 2 weeks and the hemodynamics were measured by use of Swan-Ganz catheterization and thermodilution method every 4 weeks. Values were expressed as mean${\pm}$standard deviation. Result: 4(20%) dogs died due to heart failure. Left ventricular end-diastolic volume at the 4 stages were 40.8${\pm}$7.4, 82.1${\pm}$21.1, 59.9${\pm}$7.7 and 46.5${\pm}$6.5ml. Left ventricular end-systolic volume showed the same trend. Ejection fractions were 50.6${\pm}$4.1, 17.5${\pm}$5.8, 36.3${\pm}$7.3, and 41.5${\pm}$2.4%. Blood pressure and heart rate showed no significant changes. Pressures of central vein, right ventricle, pulmonary artery, and pulmonary capillary wedge showed significant increase during the heart failure period, normalizing at the end of recovery period. Stroke volumes were 21.5${\pm}$8.2, 12.3${\pm}$3.5, 17.9${\pm}$4.6, and 15.5${\pm}$3.4ml. Blood norepinephrine level was 133.3${\pm}$60.0pg/dL at the baseline and 479.4${\pm}$327.3pg/dL at the heart failure stage(p=0.008). Conclusion: Development of tachycardia induced heart failure model is of high priority due to ready availability and reasonable amenability to measurements. Recovery trajectory after cessation of tachycardia showed reduction of cardiac dilatation and heart function. Application of new surgical procedures during the recovery period could decrease animal mortality.

• Journal of Chest Surgery
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• v.36 no.9
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• pp.646-650
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• 2003

Many surgical techniques for ischemic mitral regurgitation (IMR) have been used with their excellent results and advantages. Here, we report our simple posterior annuloplasty techniques using vascular graft strip with their early results. Material and Method: Twenty two patients (13 male) underwent the operations for IMR (excluding the papillary muscle rupture) from December 2001 to January 2003. Preoperative risk factors were low ejection fraction (<35％, n=9), hypertension (n=13), diabetes (n=9), and renal failure (Cr>2.5, n=4). The wide dissection beneath the both vena cavae and interatrial groove after bicaval cannulation enabled the easy exposure of mitral valve even in the small left atrium. After eight or nine interrupted sutures in posterior annulus for anchoring the 6 mm width vascular graft strip, symmetric (n=8) or asymmetric (n=14) annuloplasty were done. Combined surgeries were CABG (n=21), Dor procedures (n=3), tricuspid valve annuloplasty (n=1), Maze operation (n=1), and aorto-right subclavian artery bypass (n=1). Result: Except for one surgical mortality, all the patients were doing well and the mean grade of regurgitation was decreased from 2.95 to 0.88, however the ejection fraction had not changed significantly just before discharge. Post-operative valve function evaluated before discharge revealed no residual regurgitation in 8 (including 1 patient with mild stenosis due to over reduction), minimal in 11, mild in 2, and mild to moderate regurgitation in 1. One patient who had ischemic cardiomyopathy and renal failure died of the arrhythmia during the hemodialysis. Conclusion: These observations suggest that the annuloplasty with vascular graft strip could be a safe and cost effective techniques for ischemic mitral regurgitation. However, the long term evaluation for the mitral valve function should be defined for the final conclusion.

• Tuberculosis and Respiratory Diseases
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• v.47 no.1
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• pp.77-89
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• 1999

Background: High-dose chemotherapy is increasingly employed in many refractory malignant diseases. This therapy has been reported to increase response rate and survival benefits but it is also associated with higher treatment-related morbidity and mortality. We evaluated clinical characteristics and course of the pulmonary toxicity following high-dose chemotherapy with peripheral blood stem cell transplantation. Methods: Ninety-seven patients who had received high-dose chemotherapy with peripheral blood stem cell transplantation were evaluated. Five patients who developed lung lesions which were not related to infection nor primary malignant disease underwent transbronchial lung biopsy. The patients' clinical characteristics, treatments, and prognosis were reviewed retrospectively. Results: Five patients(5.1%) developed idiopathic pneumonia syndrome. The high dose chemotherapy regimens employed were cyclophosphamide, BCNU, and cisplatin in 3 cases, one case of BCNU, etoposide, Ara-C, and cyclophosphamide combination, and a regimen consisting of BCNU, etoposide, Ara-C, and melphalan. The total dose of BCNU used was 300-400 mg/$m^2$ and that of cyclophosphsmide was 6,000 mg/$m^2$. All of 5 patients received radiation therapy before this treatment. After an average duration of 14 weeks (4-26 weeks) of high-dose chemotherapy, patients developed cough, dyspnea and fever. The chest X-rays showed bilateral diffuse infiltration in 3 cases and the focal infiltration in the other 2 cases. All the patients received corticosteroid therapy as a treatment for the lung lesions. Two of them progressed to acute respiratory distress syndrome and died. Three patients recovered without residual lung lesion but one of them died of dilated cardiomyopathy. Conclusion: High-dose chemotherapy with peripheral blood stem cell transplantation especially which containing BCNU regimen may develop idiopathic pneumonia syndrome related to pulmonary toxicity and corticosteroid therapy may be bel1eficial in some cases.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.68-74
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• 2005

Purpose : There is growing use of continuous renal replacement therapy(CRRT) for pediatric patients, but reports about the use and outcome of CRRT in children is rare in Korea. We report our experiences of CRRT in critically ill pediatric patients. Methods : We reviewed the medical records of 23 pediatric patients who underwent CRRT at Asan Medical Center between May 2001 and May 2004. We evaluated underlying diseases, clinical features, treatment courses, CRRT modalities and outcomes. Results : Ages ranged from three days to 16 years with a median of five years. Patients weighed 2.4 to 63.9 kg(median 23.0 kg; 10 patients ${\leq}20kg$). The underlying diseases were malignancy(nine cases), multiple organ dysfunction syndrome(five cases), hyperammonemia(four cases), acute renal failure associated with liver failure(three cases), dilated cardiomyopathy(one case) and congenital nephrotic syndrome(one case). Pediatric Risk of Mortality(PRISM) III score was $17.6{\pm}7.6$ and the mean number of failing organs was $3.0{\pm}1.7$. Duration of CRRT was one to 27 days(median : nine days). Eleven patients(47.8%) survived. Chronic renal failure developed in two cases, intracranial hemorrhage in one case, and chylothorax in one case among the survivors. PRISM III score and the number of vasopressor before the start of CRRT was significantly lower in the survivors($12.7{\pm}4.2$ and $0.9{\pm}1.1$) compared with nonsurvivors($22.1{\pm}7.8$ and $2.4{\pm}1.4$)(P<0.05). Conclusion : CRRT driven in venovenous mode is an effective and safe method of renal support for critically-ill infants and children to control fluid balance and metabolic derangement. Survival is affected by PRISM III score and the number of vasopressors at the initiation of CRRT.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.392-398
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• 2011

Background: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was $72{\pm}37$ months (range: 4~160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.876-882
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• 2003

Purpose : To find out the myocardial protective effect of cardioxane for the myocardial damage by doxorubicin. Methods : Using Eighteen rabbits(2.0-3.2 kg), doxorubicin($30mg/m^2$) was injected intravenously once a week in group I(12 rabbits) and cardioxane($600mg/m^2$) was injected at 20-30 minutes before doxorubicin administration in group II(6 rabbits). After this, we operated on the rabbits when the total cumulative dose of doxorubicin was reached at 210, 240, 270 and $300mg/m^2$ and observed the degree of myocardial damage with light and electronic microscope. Results : In group I, rabbits with less than $210mg/m^2$ of total cumulative dose of doxorubicin, there was no definite myocardial damage but with $240mg/m^2$, focal degenerative change was observed and with $300mg/m^2$, severe degenerative change was detected with light microscopic examination. With electronic microscope, rabbits with less than $180mg/m^2$ of total cumulative dose of doxorubicin in group I, there was no evidence of myocardial damage. In $210mg/m^2$, focal degenerative change was detected. With $240mg/m^2$, degenerative change was much more advanced and with $300mg/m^2$, severe degenerative change was detected. In group II, no definite myocardial damage was observed even though the total cumulative dose of doxorubicin reached $300mg/m^2$, but with $360mg/m^2$, there was a focal area where myocardial fibers were somewhat decreased, but it's difficult to say whether these decrement were due to adriamycin in the electronic microscopic examination. Conclusion : Cardioxane have a good protective effect for the doxorubicin induced cardiomyopathy and it will be used safely in pediatric cancer patients.

• 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.43 no.1
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• pp.20-24
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• 2010

Background: Fulminant myocarditis is a rare, but life threatening condition. Its prognosis is related with proper management in the acute phase. A cardiopulmonary support device can be very useful in this phase. We report on our experiences with managing acute fulminant myocarditis with a cardiopulmonary support (CPS) device. Material and Method: We reviewed retrospectively 9 patients who had a CPS device used for their fulminant myocarditis between September, 2006 and October, 2008. A Capiox emergency bypass system (Terumo Inc, Tokyo, Japan) was percutaneously inserted in all the patients. Upon implantation, all the patients were in cardiogenic shock because of ventricular arrhythmia or severe left ventricular dysfunction. The mean left ventricular ejection fraction (EF) was $20{\pm}6%$ according to transthoracic echocardiography. Result: 3 patients died despite CPS. The CPS was bridged to a transplanted heart in one patient. The rest were successfully explanted after a mean time of $107{\pm}70$ hours of running. The mean EF after discharge was $56{\pm}7%$ without dilated cardiomyopathy. Conclusion: Fulminant myocarditis can be fatal, but its prognosis is excellent if these patients receive proper, timely treatment. A cardiopulmonary support device can be very useful in this acute period. However, the implantation and management protocol of cardiopulmonary support are not yet settled. Further study is necessary to lower the complications of cardiopulmonary support for patients with fulminant myocarditis.

• Nuclear Medicine and Molecular Imaging
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• v.43 no.6
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• pp.519-525
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• 2009

Purpose: F-18 fluorodeoxyglucose (FDG) uptake of myocardium is influenced by various factors. Increased glycolysis, and subsequent increased F-18 FDG uptake has been reported in ischemic cardiomyopathy. However, clinical significance of incidentally found myocardial F-18 FDG uptake has not been clarified. We retrospectively reviewed the degree and pattern of myocardial uptake in patients without history of ischemic heart disease who underwent torso F-18 FDG PET/CT for evaluation of neoplastic disease. Materials and Methods: From January 2005 to June 2009, 77 patients who underwent F-18 FDG PET/CT and Tc-99m sestamibi stress/rest SPECT within 3 months were enrolled. Results: Of 77 patients, 55 (71.4%) showed increased F-18 FDG uptake in the myocardium. In this population, 40 showed uniform uptake pattern, while 15 showed focal uptake. In patients with uniform uptake, 17 showed decreased uptake in the septum without perfusion defect on myocardial SPECT. Remaining 23 patients showed uniform uptake, with 1 reversible perfusion defect and 1 fixed perfusion defect. In 15 patients with focal uptake, 9 showed increased F-18 FDG uptake in the base, and only 1 of them showed reversible perfusion defect on myocardial SPECT. In the remaining 6 focal uptake group, 4 had reversible perfusion defect in the corresponding wall, and 1 had apical hypertrophy. Conclusion: We demonstrated that septal defect pattern and basal uptake pattern in the myocardium may represent normal variants. Focal myocardial uptake other than normal variants on oncologic torso F-18 FDG PET/CT with routine fasting protocol may suggest ischemic heart disease, thus further evaluation is warranted.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.135-141
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• 2014

Objectives: Fabry disease (FD) is a lysosomal storage disease caused by the inappropriate accumulation of globotriaosylceramide (Gb3) in tissues due to a deficiency in the enzyme ${\alpha}$-galactosidase A. Hypertrophic cardiomyopathy is one of the chronic complications of FD. We tried to evaluate the prevalence of Fabry disease in the Korean patients with left ventricular hypertrophy (LVH). Methods: A total of 257 patients with LVH were recruited and they were 172 males (mean 56 years, range 30-81 years) and 84 females (mean 66 years, range 45-85 years). Urinary Gb3 was used to screen FD by high performance liquid chromatography-tandem mass spectrometry. Confirmatory tests were done by alpha-galactosidaseA activity using fluorometric assay and by GLA mutation analysis using sequencing. Results: Four patients were screening positive by urinary Gb3 analysis (cutoff, 25 ug/mmol creatinine). But, one female patient was diagnosed with FD confirmed by enzyme analysis in leukocytes as well as by genetic analysis (1/257 patients, 0.4%). She showed 54.3 ug/mmoL creatinine of Gb3 and 15.5 nmole/hr/mg protein (reference range, $55.2{\pm}12.7nmole/hr/mg$ protein) of alphagalactosidase A activity. And she had a heterozygous GLA mutation of c.796G>A (p.D266N). Her daughter was found to be a carrier for FD confirmed by GLA mutation analysis. Asymptomatic carrier showed 25.5ug/mmol creatinine of Gb3 and 42.5 nmole/hr/mg protein (reference range, $55.2{\pm}12.7nmole/hr/mg$ protein) of alpha-galactosidase A activity. Conclusions: The prevalence of FD in Koran patients with LVH was detected as 0.4%. Although the prevalence seems to be low, screening studies are of great importance for detecting hidden cases as well as for identifying other effected family members.