• Title/Summary/Keyword: Endomyocardial fibrosis

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Endomyocardial Fibrosis -A case report- (심내막 심근섬유증)

  • 최창휴;박이태;박성식;류재욱;김재현;김삼현
    • Journal of Chest Surgery
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    • v.32 no.3
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    • pp.310-314
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    • 1999
  • Endomyocardial fibrosis(EMF) is an unusual type of cardiomyopathy characterized by a restriction to the ventricular filling and an obliteration of the inflow portion in the ventricular cavity by a fibrosis and often by a thrombus formation. The atrioventricular valve may be involved, resulting in an atrioventricular valvular regurgitation. The only known effective treatments are endomyocardiectomy and replacement of regurgitant AV valves. We report the experience of a case of EMF which required surgical management.

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Cardiac Behçet's Disease Presenting with Right Ventricular Endomyocardial Fibrosis and Intracardiac Thrombosis: a Case Report

  • Choi, Eun Ji;Kim, Min Sun;Koo, Hyun Jung;Song, Jae-Kwan;Song, Joo Seon;Kang, Joon-Won;Yang, Dong Hyun
    • Investigative Magnetic Resonance Imaging
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    • v.25 no.4
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    • pp.332-337
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    • 2021
  • Behçet's disease is a chronic inflammatory disorder involving vessels of various sizes and organs, including the skin, joints, gastrointestinal tract, lungs, and cardiovascular system. The etiology of Behçet's disease is unclear, and clinical diagnosis is important in the absence of definitive laboratory or pathological findings diagnostic of Behçet's disease. Cardiac involvement is rare but might present as endocarditis, myocarditis, pericarditis, or intracardiac thrombosis. This report presents a case of Behçet's disease involving the heart in a 22-year-old man with unusual manifestations of right ventricular fibrosis and intracardiac thrombosis. Cardiac magnetic resonance imaging revealed multiple intracardiac thrombi and delayed diffuse subendocardial enhancement involving the right ventricle. No peripheral eosinophilia was detected. Endomyocardial biopsy showed mixed inflammatory cell infiltrates. Based on the patient's clinical history of oral ulcer and arthritis, a diagnosis of Behçet's disease was made considering the clinical, radiological, and histological findings. Intracardiac thrombi and endomyocardial fibrosis are rare manifestations of Behçet's disease, and the diagnosis is often a clinical challenge. Early diagnosis is important for appropriate management. Behçet's disease should be considered in the differential diagnosis of patients with intracardiac thrombosis and endomyocardial fibrosis of the right chamber.

Bidirectional Cavopulmonary Shunt for Isolated Right Ventricular Endomyocardial Fibrosis (우심실에 생긴 심근내막섬유증에서 시행한 양 방향성 상대정맥-폐동맥 단락 수술)

  • 서영준;이덕헌;박남희;최세영;유영선
    • Journal of Chest Surgery
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    • v.36 no.7
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    • pp.523-526
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    • 2003
  • A 44-year-old man had been admitted for dyspnea on exertion and abdominal distension. The echocardiography revealed abnormal mass in right atrium and tricuspid valve stenosis with right ventricular obliteration. The operation was performed with mass removal, enlargement of tricuspid valve opening, and right ventricular endocardiectormy. And then, atrial septal defect was made due to inadequate right ventricular volume. The patient's symptom was improved and he discharged without events. The endomyocardial fibrosis was diagnosed with microscopic examination. Eighteen months later, the patient was readmitted due to aggravated dyspnea and cyanosis. The right ventricular obliteration was progressed and pulmonary blood flow was severely decreased in follow up echocardiography. Palliative bidirectional cave-pulmonary shunt was performed due to functional single ventricle. The dyspnea and cyanosis was markedly improved. Bidirectional cavo-pulmonary shunt for advanced and isolated right ventricular endomyocardial fibrosis provided effective palliation at early postoperative period, However, long-term follow up is mandatory.

Triptolide improves myocardial fibrosis in rats through inhibition of nuclear factor kappa B and NLR family pyrin domain containing 3 inflammasome pathway

  • Shen, Jianyao;Ma, Hailiang;Wang, Chaoquan
    • The Korean Journal of Physiology and Pharmacology
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    • v.25 no.6
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    • pp.533-543
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    • 2021
  • Myocardial fibrosis (MF) is the result of persistent and repeated aggravation of myocardial ischemia and hypoxia, leading to the gradual development of heart failure of chronic ischemic heart disease. Triptolide (TPL) is identified to be involved in the treatment for MF. This study aims to explore the mechanism of TPL in the treatment of MF. The MF rat model was established, subcutaneously injected with isoproterenol and treated by subcutaneous injection of TPL. The cardiac function of each group was evaluated, including LVEF, LVFS, LVES, and LVED. The expressions of ANP, BNP, inflammatory related factors (IL-1β, IL-18, TNF-α, MCP-1, VCAM1), NLRP3 inflammasome factors (NLRP3, ASC) and fibrosis related factors (TGF-β1, COL1, and COL3) in rats were dete cted. H&E staining and Masson staining were used to observe myocardial cell inflammation and fibrosis of rats. Western blot was used to detect the p-P65 and t-P65 levels in nucleoprotein of rat myocardial tissues. LVED and LVES of MF group were significantly upregulated, LVEF and LVFS were significantly downregulated, while TPL treatment reversed these trends; TPL treatment downregulated the tissue injury and improved the pathological damage of MF rats. TPL treatment downregulated the levels of inflammatory factors and fibrosis factors, and inhibited the activation of NLRP3 inflammasome. Activation of NLRP3 inflammasome or NF-κB pathway reversed the effect of TPL on MF. Collectively, TPL inhibited the activation of NLRP3 inflammasome by inhibiting NF-κB pathway, and improved MF in MF rats.

Clinical Analysis of 622 Cases of Open Heart Surgery (1986 년 개심술 622례에 대한 임상적 고찰)

  • 박표원
    • Journal of Chest Surgery
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    • v.20 no.3
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    • pp.489-497
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    • 1987
  • Six hundred and twenty two cases of open heart surgery were performed at Sejong General Hospital in 1986. And also, 117 cases of non open heart cardiac surgery had been performed during same period. Among the 622 open heart cases, 548 were congenital cardiac diseases and 74 were acquired heart diseases. In congenital heart patients, 422 were acyanotic and 126 were cyanotic. There were 52 cases of infant open heart Surgery below 12 months. Acyanotic group were consisted of 314 VSD, 66 ASD, 13 AVSD, 9 PDA, 8 ASD + PS, 4 AS, and 8 other rare cardiac cases. And cyanotic group were consisted of 84 TOF, 15 DORV, 5 Trilogy, 4 Ebstein`s anomaly, 3 PS + TR, 3 TGA, 3 TAPVR, 3 Pulmonary atresia and 6 other rare cardiac diseases. Majority of the acquired heart cases were valvular heart diseases. And there were also 4 cardiac myxoma and one endomyocardial fibrosis in acquired heart disease group. The operative results were as follows: Overall operative mortality, 5.3%: acyanotic 2.4%: cyanotic 15.8% and acquired heart disease, 8.5%.

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Early Stage Loeffler's Endocarditis Detected by Transthoracic Echocardiography

  • Kang, Min Kyu;Park, Won Jong;Jung, Sung Yun;Kim, Su Mi;Kwon, Tae Hun;Ryu, Young Ha;Son, Jang Won;Shin, Dong Gu
    • Journal of Yeungnam Medical Science
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    • v.29 no.2
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    • pp.118-120
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    • 2012
  • Loeffler's endocarditis involves progressive eosinophilic infiltration of the endocardium, which leads to apical thrombotic obliteration of the ventricle and endomyocardial fibrosis, that may finally represent a characteristic feature of restrictive cardiomyopathy. This paper presents a case of a 44-year-old male with symptoms of dyspnea and peripheral hypereosinophilia, who was diagnosed with early stage Loeffler's endocarditis via multicardiac imaging modalities.

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