• Investigative Magnetic Resonance Imaging
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• v.23 no.2
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• pp.100-113
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• 2019

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.523-528
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• 2005

Background: Investigation of the change of ventricular function and mitral regurgitation after surgical repair of patient with anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is key issue for the better surgical outcome. Material and Method: From April 1986 to July 2002, 12 patients presented with ALCAPA. The median age at repair was 4 months. Surgical methods included left coronary artery transfer to the aorta (10), Takeuchi procedure (1), saphenous vein free graft bypass (1). Mitral valve was repaired in 1. Result: There were 2 hospital death $(16.7\%)$. The mean follow-up period was $7.1\pm4.1$ years (range, 7 months to 13 years). Four patients required postoperative circulatory assist for $2.2\pm1.1$ days and one needed left ventricular assist device (LVAD) for 1day. Postoperative echocardiography demonstrated significant improvements in mean fractional shortening $(33.4\pm9.1\%\;vs\;17.7\pm9.6\%,\;n=10,\;p<0.05);$ left ventricular end diastolic dimension $(33.4\pm7.3\;mm\;vs\;44.8\pm7.0\;mm,\;n=10,\;p<0.05)$ and systolic dimension $(22.2\pm7.5\;mm\;vs\;33.4\pm7.9\;mm,\;n=10,\;p<0.05)$. Severities of mitral regurgitation decreased in all survivors at 1st and 4th year follow-up echocardiography. There were 2 reoperation due to residual MR and right ventricular outflow obstruction (Takeuchi case). Conclusion: Anatomic repair of anomalous left coronary artery from the pulmonary artery offered an excellent surgical results, especially in terms of the recovery of left ventricle function and mitral regurgitation. However, preoperative indications for mitral procedure is to be evaluated.

• Journal of Chest Surgery
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• v.26 no.1
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• pp.36-43
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• 1993

Sixty four children [aged 2 days to 9 years] , 58 with complete transposition of the great arteries, 5 with Taussig-Bing double outlet right ventricle, and 1 with double outlet left ventricle plus left ventricular type single ventricle, have undergone anatomic correction from November 1987 to August 1992. Eleven underwent previous operations: pulmonary artery banding[7], modified Blalock-Taussig shunt[2], coarctoplasty[2], aortic arch reconstruction[1] . Of 58 patients with TGA, Type A coronary arteries of Yacoub were seen in 50[86%]. U-shaped coroanry arterial flaps were transfered to the neoaorta using trap door technique, and neopulmonary arterial tract was constructed using glutaraldehyde fixed autopericardium with Lecompte maneuver. There were 18 hospital deaths [28.1%] with no late mortality. Mean follow-up of 20.4\ulcorner11.9 months were achieved in all survivors. Postoperative cardiac catheterizations were done in 14 cases. Mean pressure gradients of pulmonary and aortic outflow tract were 15.0 $\pm$2.6 and 4.2$\pm$1.4mmHg, mild aortic valve insufficiencies were found in 2, and mean cardiac index was 5.18$\pm$0.19 L/min/M2. We conclude that we should continue anatomic correction for the complex congenital heart anomalies with the malposition of the great arteries because myocardial function seems to be well preserved, though we are still on the learning curve.

• The Korean Journal of Nuclear Medicine
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• v.28 no.1
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• pp.37-43
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• 1994

The coronary collateral vessels have revealed their significance in terms of reduction of infarct size, preservation left ventricular function, and prevention of left ventricular aneurysm in patients with myocardial infarction. The purpose of this study were to evaluated the relation between collateral circulation and $^{99m}Tc$-MIBI Heart SPECT in patient with acute myocardial infarction and their clinical significance. The fifty six MI patients with antegrade TIMI perfusion grade 0 and 1 were studied. The patients were classified into two groups; Group I inclueded 30 patients with grade 2, 3 Collateral flow. Group II inclueded 26 patients with grade 0, 1 Collateral flow. Collateral filling were graded from 0 to 3: 0- none, 1- Filling of side branch only, 2- Partial filling of the epicardial segment, 3- Complete filling of epicardial segment. Clinical variables, left ventricular function, $^{99m}Tc$-MIBI Heart SPECT were analyzed with angiographic finding. Results were following: 1) Collateral visualization was found to be greater in patient with involvement of right coronary artery (RCA). The collateral development site of infarct related artery was RCA 15 cases, left anterior descending artery (LAD) 10 cases, left circumflex artery (LCX) 5 cases, and the collateral circulation from LAD to RCA was 13 cases (40.6%). 2) There was a tendency to be decreased in peak CK activity with group I. 3) The presence of good collateral channels was more frequently $^{99m}Tc$-MIBI reversible perfusion defect (83.4% vs 15.3%, p<0.05). 4) No differences of left ventricular end diastolic volume (LVEDV), left ventricular end systolic volume (LVESV), ejection fraction (EF) were noted between group I and group II. The presence of good collateral channels did affect the frequency of occurrence of $^{99m}Tc$-MIBI reversible perfusion defect.

• Korean Journal of Veterinary Research
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• v.38 no.1
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• pp.29-42
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• 1998

$Mg^{2+}$ is one of the most abundant divalent cations in mammalian body(0.2~1.0mM) and the important physiological roles are : first, the cofactor of many enzyme activities, second, the regulator of glycolysis and DNA synthesis, third, the important role of bioenergetics by regulating of phosphorylation, fourth, the influence of cardiac metabolism and function. In this work we have investigated the regulation of the $Mg^{2+}$ induced by ${\alpha}_1-adrenoceptor$ stimulation in perfused guinea pig hearts and isolated myocytes. The $Mg^{2+}$ content of the perfusate or the supernatant was measured by atomic absorbance spectrophotometry. The elimination of $Mg^{2+}$ in the medium increased the force of contraction of right ventricular papillary muscles, and the left ventricular pressure. Phenylephrine also enhanced the force of contraction in the presence of $Mg^{2+}-free$ medium. ${\alpha}_1-Agonists$ such as phenylephrine and methoxamine were found to induce $Mg^{2+}$ efflux in both perfused hearts and myocytes. These effects were blocked by prazosin, an ${\alpha}_1-adrenoceptor$ antagonist. The $Mg^{2+}$ influx could also be induced by phenylephrine and R59022, a diacylglycerol kinase inhibitor. In the presence of protein kinase C(PKC) inhibitors, phenylephrine produced an increase in $Mg^{2+}$ efflux from perfused hearts. Furthermore, $Mg^{2+}$ efflux by phenylephrine was amplified by phorbol 12-myristate 13-acetate(PMA). This enhancement of $Mg^{2+}$ efflux by PMA was blocked by prazosin in perfused hearts. By contrast, the $Mg^{2+}$ influx could be induced by verapamil, nifedipine, ryanodine in perfused hearts, but not in myocytes. $W^7$, a $Ca^{2+}$/calmodulin antagonist, completely blocked the phenylephrine-induced $Mg^{2+}$ efflux in perfused hearts. In conclusion, $Mg^{2+}$ is responsible for the cardiac activity associated with ${\alpha}_1-adrenoceptor$ stimulation. The mobilization of $Mg^{2+}$ is decreased or increased by ${\alpha}_1-adrenoceptor$ stimulation in guinea pig hearts. These responses may be related specifically to the respective pathways of signal transduction. A decrease in $Mg^{2+}$ efflux by ${\alpha}_1-adrenoceptor$ stimulation in hearts can be through PKC dependent and intracellular $Ca^{2+}$ levels.

• Journal of Veterinary Clinics
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• v.30 no.6
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• pp.468-472
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• 2013

A 12-year-old intact female mixed dog (weighing 6.2 kg) was referred with primary complaints of severe abdominal distension, persistent coughing and exercise intolerance. Diagnostic studies found ascites, jugular distension, loud left and right apical systolic (grade 4/6) murmur, generalized cardiomegaly with caudal vena cava distension and left atrial dilation. Echocardiographic findings were consistent with degenerative mitral and tricuspid valve endocardiosis and bi-ventricular congestive heart failure. There was also a left to right shunting patent foramen ovale. The LV systolic function is depressed relative to the degree of volume overload. Based on diagnostic findings, this case was diagnosed as PFO complicated with mitral and tricuspid valve endocardiosis with ISACHC IIIa heart failure. The dog was treated with furosemide (2 mg/kg, q12hr, PO), sildenafil (1 mg/kg, q8hr, PO), pimobendan (0.3 mg/kg, q12hr, PO), enalapril (0.5 mg/kg, q12hr, PO) and spironolactone (1 mg/kg, q12hr). The clinical signs were gradually improved after medical therapy.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.482-491
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• 2004

Background: The xenogenic or allogenic valves after in Vitro repopulation with autologous cells or in vivo repo-pulation after acellularization treatment to remove the antigenicity could used as an alternative to synthetic polymer scaffold. In the present study, we evaluated the process of repopulation by recipient cell to the acellu-larized xenograft treated with NaCl-SDS solution and grafted in the right ventricular outflow tract. Material and Method: Porcine pulmonary valved conduit were treated with. NaCl-SDS solution to make the grafts acellularized and implanted in the right ventricular outflow tract of the goats under cardiopulmonary bypass. After evaluating the functions of pulmonary valves by echocardiography, goats were sacrificed at 1 week, 1 month, 3 months, 6 months, and 12 months after implantation, respectively. After retrieving the implanted valved conduits, histopathologic examination with Hematoxylin-Eosin, Masson' trichrome staining and immunohistochemical staining was performed. Result: Among the six goats, which had been implanted with acellularized pulmonary valved conduits, five survived the expected time period. Echocardiographic examinations for pulmonary valves revealed good function except mild regurgitation and stenosis. Microscopic analysis of the leaflets showed progressive cellular in-growth, composed of fibroblasts, myofibroblasts, and endothelial cells, into the acellularized leaflets over time. Severe inflammatory respon-se was detected in early phase, though it gradually decreased afterwards. The extracellular matrices were regenerated by repopulated cells on the recellularized portion of the acellularized leaflet. Conclusion: The acellularized xenogenic pulmonary valved conuits were repopulated with fibroblasts, myofibroblasts, and endothelial cells of the recipient and extracellullar matrices were regenerated by repopulted cells 12 months after the implantation. The functional integrity of pulmonary valves was well preserved. This study showed that the acellularized porcine xenogenic valved conduits could be used as an ideal valve prosthesis with long term durability.

• The Korean Journal of Physiology and Pharmacology
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• v.15 no.4
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• pp.189-194
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• 2011

ATP-sensitive $K^+$ channels ($K_{ATP}$) are major component of preventing ischemia-reperfusion injury. However, there is little information regarding to the expressional difference of $K_{ATP}$ and its function between left and right ventricles. In this study, we measured the lactate dehydrogenase release of rabbit heart slices in vitro and determined the difference of the $K_{ATP}$ expression at the both ventricles by measuring the level of $K_{ATP}$-forming Kir6.2 (OcKir6.2) mRNA using in situ hybridization. The hearts were preconditioned with 15 min hypoxia and reoxygenated for 15 min before a hypoxic period of 60 min, followed by reoxygenation for 180 min. With hypoxic preconditioning (100% $N_2$) with 15 min, left ventricles (LV) showed higher release of LDH comparing with right ventricles (RV). Adding $K_{ATP}$ blocker glibenclamide ($10{\mu}M$) prior to a hypoxic period of 60 min, hypoxic preconditioning effect of RV was more abolished than LV. With in situ hybridization, the optical density of OcKir6.2 was higher in RV. Therefore, we suggest that different $K_{ATP}$ expression between LV and RV is responsible for the different response to hypoxia and hypoxic preconditioning of rabbit hearts.

• 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.38 no.9 s.254
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• pp.609-615
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• 2005

Background: Some controversy still exists concerning the operative indications of coronary fistulas. Nevertheless, a short-term and long-term outcomes are excellent with surgical interventions. In this study, we assessed our surgical results on this disease entity during the last 20 years. Anatomic diversity was described as well. Material and Method: From April 1986 to March 2005, 20 patients with coronary fistulas underwent surgical correction in Seoul National University Children's Hospital. Their medical records were reviewed retrospectively. Result: Twelve patients ($60\%$) were asymptomatic prior to surgery. All had electrocardiogram and echocardiogram and all but 3 had coro-nary angiogram preoperatively. Anatomically, none of them had two or more coronary fistulas. The sites of origin were left coronary system in 11 patients and right in 9. The draining sites were right ventricle in 11, right atrium in 3, left ventricle in 3, main pulmonary artery in 2, and superior vena cavae in 1. All of the involved, the coro-nary arteries were dilated or aneurismal. In 1 case, there was atherosclerotic change but no ischemic evidence in preoperative electrocardiogram. Operative techniques included external obliteration (13), internal obliteration (5), and both (2). External obliteration was done by ligation of the fistulous tract only in T patients, by fstula ligation plus plication in 3 and by plication or patch closure via fistulotomy in 3. There was no operative mortality. All of postoperative morbidities including transient sinus arrhythmia (2), complete atrioventricular block (1), decreased left ventricular function (2), ventricular tachycardia (1), pericarditis (1), and seizure (1) improved on discharge. The mean follow-up was 55.1$\pm$50.2 months (4.0 months${\~}$18.0 years) and there were no recurrences of fistula. There was 1 second operation for aortic root aneurysm, which developed after external patch closure of right coronary fistula. Conclusion: We demonstrated here that coronary fistulas can be cured with excellent clinical outcome and low operative risk under precise diagnosis. Understanding the anatomic diversity will help to construct surgical plans.