• Title/Summary/Keyword: cardiomyopathy

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Idiopathic Hypertropic Subaortic Stenosis with Mitral Regurgitation -Report of 3 Cases- (특발성 비후성 대동맥판하 협착증 및 승모판 폐쇄부전증 -3례 보고-)

  • 강청희
    • Journal of Chest Surgery
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    • v.27 no.4
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    • pp.313-317
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    • 1994
  • Surgical treatment is possible for the obstructive form of hypertrophic cardiomyopathy and transaortic left ventricular septal myectomy and myotomy has been the procedure of choice. If coexisting intrinsic mitral valve disease exists, mitral valve replacement has been performed. But abnormal systolic anterior motion of anterior mitral leaflet[SAM] with intrinsic normal mitral valve disease is the typical feature of IHSS and we prefer not to replace mitral valve. 3 patients underwent transaortic myotomy and myectomy for IHSS with mitral regurgitation. 2 patients of them have coexisting intrinsic mitral valve diseases such as mitral valve vegetation and chorda rupture. Concomittent mitral valve replacement were performed. 1 patient shows SAM of mitral anterior leaflet but has intrinsic normal mitral valve morphologically and transepicardial echocardiogram and direct monitoring of pressure gradient during the operative procedure gives better information for subsided mitral regurgitation. Post operative course during the 12 months follow-up was uneventful.

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Cardiomyopathies in children

  • Hong, Young Mi
    • Clinical and Experimental Pediatrics
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    • v.56 no.2
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    • pp.52-59
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    • 2013
  • Cardiomyopathy (CMP) is a heterogeneous disease caused by a functional abnormality of the cardiac muscle. CMP is of 2 major types, dilated and hypertrophic, and is further classified as either primary or secondary. Secondary CMP is caused by extrinsic factors, including infection, ischemia, hypertension, and metabolic disorders. Primary CMP is diagnosed when the extrinsic factors of secondary CMP are absent. Furthermore, the World Health Organization, American Heart Association, and European Cardiology Association have different systems for clinically classifying primary CMP. Primary CMP is rare and associated with a family history of the disease, implying that genetic factors might affect its incidence. In addition, the incidence of CMP varies widely according to patient ethnicity. Genetic testing plays an important role in the care of patients with CMP and their families because it confirms diagnosis, determines the appropriate care for the patient, and possibly affects patient prognosis. The diagnosis and genetic identification of CMP in patients' families allow the possibility to identify novel genes that may lead to new treatments. This review focuses on the epidemiology, pathophysiology, diagnosis, and treatment of CMP, with the aim of providing pediatricians with insights that may be helpful in the early identification and management of idiopathic CMP in children.

Evaluation of Myocardial Blood Flow and Coronary Flow Reserve Using Positron Emission Tomography (양전자방출단층촬영을 이용한 심근혈류 및 관상동맥 혈류예비능 평가)

  • Lee, Byeong-Il;Bom, Hee-Seung
    • The Korean Journal of Nuclear Medicine
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    • v.39 no.2
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    • pp.118-123
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    • 2005
  • Positron emission tomography (PET) serves as a gold standard for noninvasive in vivo measurement of myocardial blood flow (MBF) and coronary flow reserve (CFR). CFR can be defined as the ratio of maximally vasodilated MBF over its basal flow. It is an important parameter for the evaluation of functional severity of coronary stenosis and prognositification in various diseases such as dilated cardiomyopathy. $^{13}NH_3,\;H_2^{15}O,\;^{82}Rb$ are widely used radiopharmaceuticals for measuring MBF and CFR, This review introduces imaging techniques and its clinical utility. Cardiac application or PET and PET/CT is expected to be increased in near future.

Surgical Management of Cardiac Tamponade (심장압전의 외과적 치료)

  • 장재현
    • Journal of Chest Surgery
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    • v.25 no.1
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    • pp.66-72
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    • 1992
  • From March 1986 to March 1991, 29 patients were operated due to cardiac tamponade at the Department of Thoracic and Cardiovascular Surgery, Masan Koryo General Hospital. Among them, 19cases were traumatic origin and 10 were Non traumatic origin. A] Traumatic cardiac tamponade Out of 19 cases, 12 cases were resulted from penetrating injury and 7 cases from trauma. Average time interval from arrival to operation was 91 minutes[15min.~8.5hr.] in penetrating injury group. On the other hand, average time of in cases of blunt trauma was more than 3hours because of the difficulties in diagnosis. Four deaths occured in 19 cases [mortality rate: 21.1%] 3 in blunt trauma group[42.9%] and 1 in penetrating group[8.3%]. In view of our experience, the prognosis was closely correlated with injury mode, initial vital sign and mental status. There was no close correlation between prognosis and cardiac injury site. B] Non traumatic cardiac tamponade The etiologies were malignancy[4], non-spesific pericarditis[3], tuberculosis[1], pyogenic[1] and cardiomyopathy[1]. All of the cases in which performed tube pericardiostomy were the cases that showed no response to conservative treatment and repeat per-icardiocentesis. There was one posoperative death.

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Postoperative Clinical Evaluation of Mechanical Valve Replacement (기계적판막의 임상적 고찰)

  • 송인기
    • Journal of Chest Surgery
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    • v.21 no.1
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    • pp.62-69
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    • 1988
  • In the department of chest surgery of WonKwang university hospital, mechanical valve replacement was performed in 51 cases from June 1985 to September 1987. Among these, 32 cases were mitral valve replacement, 4 cases were aortic valve replacement, and 15 cases were double valve replacement. 26 cases were male and 25 cases were female and age distribution ranged from 16 years old to 63 years old. Early death within 30 days after operation was 2 cases [3.9%] and causes of death were right heart failure [1] and right ventricular wall rupture [1]. Among 49 early survivors, 2 cases of late death were developed and the causes of death were cardiomyopathy [1] and ventricular arrhythmia [1] Anticoagulant therapy was done with warfarin sodium to the level of 1.5-2 times of normal prothrombin time [20-40%] in 47 survivors. Symptomatically, 93.6% of preoperative NYHA functional class III or IV were converted to the NYHA functional class I or II during follow up.

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Iatrogenic Hemocromatosis Case in Propionic Acidemia (프로피온산 혈증 환아에서 경험한 의원성 헤모크로마토시스 I례)

  • Kim, Sook Za;Jeon, Young Mi;Song, Woong Ju
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.13 no.1
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    • pp.54-56
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    • 2013
  • Propionic acidemia is an inherited organic acid metabolic disorder. During chronic recurrent metabolic crisis, multiple blood transfusions can cause secondary hemochromatosis. We report a patient with propionic acidemia who had iron overload that resulted in liver dysfunction, cardiomyopathy and diabetes. When multiple blood transfusions are unavoidable, use of chelating agents for iron can prevent complications such as diabetes and hemochromatosis.

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Design of A Human Model of the Moving-Actuator Type Total Artificial Heart

  • Chang, Jun-Keun;Min, Byoung-Goo
    • Journal of Biomedical Engineering Research
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    • v.18 no.1
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    • pp.65-70
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    • 1997
  • A human version of Korean total artificial heart(TAM) was designed basso on the magnetic resonance imaging(MRI) data To obtain accurate measurement or human thoracic structure including the valvular sited we analyzed the dimensions of the natural heart of healthy persons and cardiomyopathy(CM) patients. The MRI findings were analyzed to measure the volume of the thoracic cavity that would be occupied by the TAM. The design upgrade of the mechanical performed was also performed with the computer aided design(CAD) system to develop a new version of Korean TAH.

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Purification and Characterization of a Ubiquitin-like System for Autophagosome Formation

  • Bae, Ju-Young;Park, Hyun-Ho
    • Journal of Microbiology and Biotechnology
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    • v.20 no.12
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    • pp.1647-1652
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    • 2010
  • Autophagy refers to the bulk degradation of cellular proteins and organelles through an autophagosome and plays a pivotal role in the development, cellular differentiation, aging, and elimination of aberrant structures. A failure of autophagy has been implicated in a growing list of mammalian disease states, including cancer and cardiomyopathy. Two ubiquitin-like systems are highly involved in autophagy, especially in the formation of autophagosomes. Here, we purified and characterized Atg7 (an E1-like enzyme), and Atg3 and Atg10 (E2-like enzymes) in order to gain an insight into the role played by ubiquitin-like systems in the formation of autophagosomes. Interestingly, we observed that Atg7 forms a homodimer to construct an active conformation, unlike other E1-like enzymes. Although Atg3 was detected as a monomer under physiological conditions, Atg10 existed in an oligomeric form, indicating that the mechanism by which Atg10 functions may differ from that of Atg3.

Diabetic Alterations in Cardiac Sarcoplasmic Reticulum $Ca^{2+}$-ATPase and Phospholamban Protein Expression

  • Lee, Hee-Ran;Cho, Yong-Sun;Park, So-Young;Kim, Young-Hoon;Kim, Hae-Won
    • Proceedings of the Korean Biophysical Society Conference
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    • 2001.06a
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    • pp.66-66
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    • 2001
  • Diabetic cardiomyopathy has been suggested to be caused by abnormal intracellular $Ca^{2+}$ homeostasis in the myocardium, which is partly due to a defect in calcium transport by the cardiac sarcoplasmic reticulum (SR). In the present study, the underlying mechanism for this functional derangement was investigated with respect to SR $Ca^{2+}$-ATPase and phospholamban (PLB, the inhibitor of SR $Ca^{2+}$-ATPase).(omitted)d)

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Effect of Ginseng Components on the Potassium Depleted Cardiomyopathic Rats and it's Mechanism of Action

  • Lee, Jong-Wook;Kim, Nak-Doo
    • Archives of Pharmacal Research
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    • v.8 no.2
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    • pp.49-57
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    • 1985
  • The effect of ginseng components on the potassium depleted cardiomyopathic rat heart was investigated. In the perfused heart experiment using Langendorff apparatus, the deterioration rate of contriactile force of potassium depleted rat heart (low potassium diet group) was faster than that of normal rat heart and ginseng components showed the ability to slow the deterioration rate of potassium depleted hearts. Both sialic acid contents in carcolemmal ghost and sialyltransferase activity of 40,000 * g subcellular fraction prepared from cardiac ventricular tissue of low potassium diet group were significantly decreased compared to those of normal group. The decrease of the sialic acid content and sialyltransferase activity in sarcolemma of low potassium diet group was inhibited when ginseng was concomitantly administered. Calcium uptake of sarcoplasmic reticulum prepared from low potassium diet group was significantly greaterthan that of normal group. Ginseng extract or total saponin showed the tendency to inhibit the increase of cacium uptake.

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