• Journal of Chest Surgery
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• v.35 no.5
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• pp.336-342
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• 2002

Background: Recently, cell transplantation has been extensively investigated to improve heart function in dysfunctional heart. This study was designed to compare the effects of smooth muscle cells (SMC) and heart cells (HC) transplantation in dilated cardiomyopathic hamsters. Material and Method: HC and SMC were isolated from heart and ductus deferens of BIO 53.58 hamsters, and cultured for transplantation. HC and SMC or culture medium were transplanted into the left ventricle of 17 weeks old adult hamsters in HC transplanted (HCTx), SMC transplantation (SMCTX), and control groups (Con) (N = 10 each). Cyclosporine (5 mg/Kg) was administered subcutaneously for HCTx. Sham operated hamsters (N=10) underwent the surgery but did not receive an injection. At 4 weeks after transplantation, heart function was evaluated in all groups using a Langendorff perfusion apparatus. Result: Histology showed severe focal myocardial necrosis in all groups. HCTx and SMCTx formed huge muscle tissue in dilated myocardium. SMCTx and HCTx had better heart function than Con and sham (p<0.01). And SMCTx had better peak systolic pressure (p<0.05) antral developed pressure (p<0.05) than HCTx. But sham and Con did not any statistical make difference. Conclusion: SMCTx and HCTx formed muscle tissue and improved ventricular function in hamsters with dilated cardiomyopathy And SMCTx showed better heart function in peak systolic pressure and developed pressure than HCTx.

• Journal of Chest Surgery
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• v.30 no.6
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• pp.613-616
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• 1997

We present a case of 58-year-old (tamale with dilated cardiomyopathy(DCMP) in whom we performed left ventricular(LV) remodeling surgery(Batista operation) to reduce the left ventricle diameter and improve left ventricular unction. The patient was admitted September 1996 with heart failure NYHA class IV. There was severe orthopnea and peripheral edema. 2-D echocardiography(Echo) showed DCMP with the ejection fraction(EF) I5%, LV end diastolic dimension(LVEDD) 80mm, mitral regurgitation(MR) grade IV, tricuspid regurgitation ('m) grade ll. Preoperative cardiac output(CO) was 1.5/L/min and cardiac index(Cl) was 1.0 L/min/m2. We proceeded with LV remodeling surgery by resection a part of LV lateral wall between both papillary muscle, from the mitral annulus to the LV apex. Size of resected LV wall was 90 $\times$ 100 $\times$ 15 mm. At the mean time, mitral valve and tricuspid valve were repaired. Postoperative 2-D Echo showed the EF 37%, LVEDD 50 mna, trivial MR, no TR. CO was 3.SL/min and Cl was 2.3 L/min/m2. Her fuctional NYHA class was 1.

• Korean Circulation Journal
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• v.54 no.8
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• pp.468-481
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• 2024

Background and Objectives: Although the clinical consequences of advanced heart failure (HF) may be similar across different etiologies of cardiomyopathies, their proteomic expression may show substantial differences in relation to underlying pathophysiology. We aimed to identify myocardial tissue-based proteomic characteristics and the underlying molecular pathophysiology in non-ischemic cardiomyopathy with different etiologies. Methods: Comparative extensive proteomic analysis of the myocardium was performed in nine patients with biopsy-proven non-ischemic cardiomyopathies (3 dilated cardiomyopathy [DCM], 2 hypertrophic cardiomyopathy [HCM], and 4 myocarditis) as well as five controls using tandem mass tags combined with liquid chromatography-mass spectrometry. Differential protein expression analysis, Gene Ontology (GO) analysis, and Ingenuity Pathway Analysis (IPA) were performed to identify proteomic differences and molecular mechanisms in each cardiomyopathy type compared to the control. Proteomic characteristics were further evaluated in accordance with clinical and pathological findings. Results: The principal component analysis score plot showed that the controls, DCM, and HCM clustered well. However, myocarditis samples exhibited scattered distribution. IPA revealed the downregulation of oxidative phosphorylation and upregulation of the sirtuin signaling pathway in both DCM and HCM. Various inflammatory pathways were upregulated in myocarditis with the downregulation of Rho GDP dissociation inhibitors. The molecular pathophysiology identified by extensive proteomic analysis represented the clinical and pathological properties of each cardiomyopathy with abundant proteomes. Conclusions: Different etiologies of non-ischemic cardiomyopathies in advanced HF exhibit distinct proteomic expression despite shared pathologic findings. The benefit of tailored management strategies considering the different proteomic expressions in non-ischemic advanced HF requires further investigation.

• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1261-1265
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• 2007

Extrarenal manifestations of hemolytic uremic syndrome (HUS) have increasingly been recognized and may be major determinants of mortality and morbidity. Although microthrombi are often found in the pulmonary and coronary circulation, apparent lung and cardiac involvement are clinically infrequent. We describe here a 10-month-old boy with HUS who developed pulmonary hemorrhage, acute respiratory distress syndrome and dilated cardiomyopathy. Complete renal as well as clinical recovery from these very uncommon complications was achieved by optimum supportive care.

• The Korean Journal of Nuclear Medicine
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• v.27 no.2
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• pp.195-202
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• 1993

$^{123}Iodine$-metaiodobenzylguanidine (MIBG) which is a norepinephrine analogue, can be used to evaluate the sympathetic innervation of the heart. In this study, cardiac imaging with $^{123}I-MIBG$ was performed in patients with 9 dilated cardiomyopathy, 2 ischemic cardiomyopathy and 1 acute myocardial infarction to evaluate the sympathetic nervous function. $^{123}I-MIBG$ imaging showed multifocal defects (8), diffuse defect (2), near non-visualization (2). The defects of MIBG scans were found to be larger and more severe on 4 hours image than 30 minutes. Heart to lung, heart to mediastinum ratios were decreased at 4 hours than those at 30 minutes. Measured LVEF values were not correlated with the severity of MIBG uptake. $^{99m}Tc-MIBI$ imaging was also performed in all patients to find the relationship with $^{123}I-MIBG$ scan. $^{99m}Tc-MIBI$ scan showed multifocal defects in 9 patients, diffuse defects in 1 patient and no defect in 2 patients. The defects are similar in size, severity and extent, but more larger and severe on $^{123}I-MIBG$ imaging. Therefore, cardiac $^{123}I-MIBG$ imaging is a useful method to evaluate the sympathetic nervous function in cardiomyopathy.

• Journal of Veterinary Clinics
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• v.23 no.3
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• pp.349-354
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• 2006

A 9-month-old, female Miniature Pinscher(MP) dog weighing 1.97kg was presented because of periodic syncopal episode for 5 months. This case was diagnosed as primary dilated cardiomyopathy based on respiratory distress history, weak femoral pulse, generalized cardiomegaly, pulmonary edema, marked dilation of left atrium(LA) and left ventricle(LV), decreased wall thickness of LV and interventricular septum(IVS), increased EPSS in echocardiography, and young age of onset in the absence of other cardiovascular disorders. The patient was stabilized by application of diuretics(Furosemide, 2 mg/kg, SC, q 1 hr) and venodilator(Nitroglycerine patch, 0.5 mg/kg, q 12 hrs). Clinical signs were improved with medical management of positive inotropic vasodilator(Pimobendan, 0.2 mg/kg, PO, q 12 hrs) and angiotensin-converting enzyme(ACE) inhibitor(benazepril, 0.5 mg/kg, PO, q 12 hrs), potassium gluconate gel(2 mEq/dog, PO, q 12 hrs) and, L-carnitine(50 mg/kg, PO, q 12 hrs). The dog still maintains stable clinical status 10 months after the first visit. We report the rare case of DCM in small breed dog, which corresponds to the diagnosis and treatment of typical DCM in large breed dog.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.329-334
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• 2008

Kwashiorkor is a syndrome of severe protein malnutrition, which manifests itself in hypoalbuminemia, diarrhea, dermatitis, and edema. It can be life-threatening due to associated immune deficiency and an increased susceptibility to infections. Kwashiorkor should be treated early with nutritional support and the control of infection. Dilated cardiomyopathy may develop during the treatment and in such cases a poor prognosis is expected. Kwashiorkor has been known as a common disease of poor countries. To date, in fact, there has been no report of kwashiorkor leading to death in technically advanced countries. We here report a fatal case of a baby girl admitted with kwashiorkor. She had been fed only with cereal grain mixed with juice, without any protein supplement, for 2 months. This diet was deficient not because of poverty, but due to the illiteracy of her parents. The patient suffered from diarrhea, whole body edema, hypothermia, and dermatitis. Laboratory findings revealed an immune-deficient state featuring leukopenia and decreased immunoglobulin. Blood and urine cultures revealed Alcaligenes Xylosoxidans growth. The patient was fed frequent small amounts of protein-containing formula and intravenous albumin and micronutrients were administered for nutritional support. She was also treated with intravenous immunoglobulin and antibiotics in order to control infection. Nevertheless, she developed dilated cardiomyopathy and multi-organ failure and died. We review this case in light of the literature.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.22-27
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• 2021

Propionic acidemia (PA) is an inherited autosomal recessive disorder, due to the deficiency of propionyl-CoA carboxylase (PCC). PCC is the enzyme which catalyzes the conversion of propionyl-CoA to D-methylmalonyl-CoA, and it is critical for the metabolism of amino acids, odd-chain fatty acids, and side chains of cholesterol. The clinical manifestations present mostly at the neonatal period with life-threatening metabolic acidosis and hyperammonemia. Here, we described a case of a 16-year-old Korean boy with late-onset PA who presented with embolic cerebral infarction due to dilated cardiomyopathy (DCMP) with left ventricular noncompaction. And he has family history of sudden cardiac death, so we performed metabolic screening and genetic tests. Elevated levels of 3-hydroxypropionic acid, methylcitric acid and propionylglycerine were detected in urine. Plasma acylcarnitine profile showed elevated propionylcarnitine (C3). Diagnosis of PA was confirmed by genetic analysis, which revealed compound heterozygous mutations, c.[1151T>G] (p.[Phe384Cys]) and c.[1228C>T] (p.[Arg410Trp]) in PCCB gene. His heart function is in improving state and the results of biochemical analysis are stable with heart failure medication and metabolic managements. We present a case of patient without episodes of metabolic decompensation who manifests DCMP as the first symptom of PA.

• Korean Journal of Radiology
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• v.25 no.6
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• pp.540-549
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• 2024

Objective: This study investigated the feasibility and prognostic relevance of threshold-based quantification of myocardial delayed enhancement (MDE) on CT in patients with nonischemic dilated cardiomyopathy (NIDCM). Materials and Methods: Forty-three patients with NIDCM (59.3 ± 17.1 years; 21 male) were included in the study and underwent cardiac CT and MRI. MDE was quantified manually and with a threshold-based quantification method using cutoffs of 2, 3, and 4 standard deviations (SDs) on three sets of CT images (100 kVp, 120 kVp, and 70 keV). Interobserver agreement in MDE quantification was assessed using the intraclass correlation coefficient (ICC). Agreement between CT and MRI was evaluated using the Bland-Altman method and the concordance correlation coefficient (CCC). Patients were followed up for the subsequent occurrence of the primary composite outcome, including cardiac death, heart transplantation, heart failure hospitalization, or appropriate use of an implantable cardioverter-defibrillator. The Kaplan-Meier method was used to estimate event-free survival according to MDE levels. Results: Late gadolinium enhancement (LGE) was observed in 29 patients (67%, 29/43), and the mean LGE found with the 5-SD threshold was 4.1% ± 3.6%. The 4-SD threshold on 70-keV CT showed excellent interobserver agreement (ICC = 0.810) and the highest concordance with MRI (CCC = 0.803). This method also yielded the smallest bias with the narrowest range of 95% limits of agreement compared to MRI (bias, -0.119%; 95% limits of agreement, -4.216% to 3.978%). During a median follow-up of 1625 days (interquartile range, 712-1430 days), 10 patients (23%, 10/43) experienced the primary composite outcome. Event-free survival significantly differed between risk subgroups divided by the optimal MDE cutoff of 4.3% (log-rank P = 0.005). Conclusion: The 4-SD threshold on 70-keV monochromatic CT yielded results comparable to those of MRI for quantifying MDE as a marker of myocardial fibrosis, which showed prognostic value in patients with NIDCM.

• Korean Journal of Radiology
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• v.24 no.5
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• pp.395-405
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• 2023

Objective: This study aimed to develop and validate models using radiomics features on a native T1 map from cardiac magnetic resonance (CMR) to predict left ventricular reverse remodeling (LVRR) in patients with nonischemic dilated cardiomyopathy (NIDCM). Materials and Methods: Data from 274 patients with NIDCM who underwent CMR imaging with T1 mapping at Severance Hospital between April 2012 and December 2018 were retrospectively reviewed. Radiomic features were extracted from the native T1 maps. LVRR was determined using echocardiography performed ≥ 180 days after the CMR. The radiomics score was generated using the least absolute shrinkage and selection operator logistic regression models. Clinical, clinical + late gadolinium enhancement (LGE), clinical + radiomics, and clinical + LGE + radiomics models were built using a logistic regression method to predict LVRR. For internal validation of the result, bootstrap validation with 1000 resampling iterations was performed, and the optimism-corrected area under the receiver operating characteristic curve (AUC) with 95% confidence interval (CI) was computed. Model performance was compared using AUC with the DeLong test and bootstrap. Results: Among 274 patients, 123 (44.9%) were classified as LVRR-positive and 151 (55.1%) as LVRR-negative. The optimism-corrected AUC of the radiomics model in internal validation with bootstrapping was 0.753 (95% CI, 0.698-0.813). The clinical + radiomics model revealed a higher optimism-corrected AUC than that of the clinical + LGE model (0.794 vs. 0.716; difference, 0.078 [99% CI, 0.003-0.151]). The clinical + LGE + radiomics model significantly improved the prediction of LVRR compared with the clinical + LGE model (optimism-corrected AUC of 0.811 vs. 0.716; difference, 0.095 [99% CI, 0.022-0.139]). Conclusion: The radiomic characteristics extracted from a non-enhanced T1 map may improve the prediction of LVRR and offer added value over traditional LGE in patients with NIDCM. Additional external validation research is required.