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Fang Wei - - 2010
Dilated cardiomyopathy (DCM) is characterized by ventricular chamber enlargement and systolic dysfunction with normal ventricular wall thickness. More recent studies have shown that the coronary microcirculation may be directly affected in cardiomyopathies. Left ventricular regional myocardial perfusion, wall motion, and metabolism abnormalities are often present in patients with DCM. These ...
Cannavale Alessandro - - 2010
This report describes a 22-year-old woman who has clinical and physiologic features of a restrictive cardiomyopathy. Magnetic resonance imaging showed myocardial delayed enhancement and interventricular septal crypts characteristic of hypertrophic cardiomyopathy (HCM). Transcatheter biopsy confirmed the diagnosis, revealing marked myocyte hypertrophy, interstitial fibrosis, and fiber disarray, which are findings consistent ...
Goswami Shyamal K - - 2010
Oxygen plays a critical role in the perpetuation and propagation of almost all forms of life. The primary site of cellular oxygen consumption is the mitochondrial electron transport chain and in addition, oxygen is also used as a substrate for various enzymes involved in cellular homeostasis. Although our knowledge of ...
Adawi Salim - - 2011
The Brockenbrough-Braunwald-Morrow sign is the paradoxical decrease in pulse pressure during the post-extrasystole beat seen in patients with hypertrophic obstructive cardiomyopathy. We present a case of intermittent left ventricular outflow tract obstruction and secondary mitral regurgitation resulting from post-extrasystolic potentiation following a premature atrial beat, demonstrating using echocardiography the mechanism ...
Brabham William W - - 2011
Takotsubo cardiomyopathy (TCM) is usually characterized by left ventricular anteroapical dysfunction in the absence of significant coronary disease commonly precipitated by an emotional or stressful trigger. Hypertrophic cardiomyopathy (HCM) is usually diagnosed on the basis of symptoms, family history, echocardiography, or by the presence of a characteristic murmur. We report ...
Arakawa Kenichiro - - 2010
Cardiomyopathy is a life-threatening condition in patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (known as MELAS). However, no effective therapy has been available until now. In the present study cardiac energetics and acute effects of L-arginine (Arg) were evaluated in MELAS patients. The 6 patients with MELAS ...
Stensæth Knut Haakon - - 2011
Abstract Objectives. Takotsubo cardiomyopathy (TTC) is a diagnostic entity that is increasingly being recognized. Data from cardiac magnetic resonance (CMR) imaging and its impact on differential diagnosis are limited. Methods and results. After 26 months, coronary angiography revealed normal coronary arteries and left ventriculography and/or echocardiography left ventricular dysfunction with ...
Konstandin Mathias H - - 2011
We present the case of a 67-year-old woman presenting with symptoms of predominantly right heart failure caused by cardiac tumor infiltration. Echocardiography showed almost complete obstruction, particularly of the right ventricular outflow tract. After complete diagnostic workup, treatment with only one course of bendamustine and rituximab resulted in complete resolution ...
Adamo Candace M CM Departments of Pharmacology, University of Washington, Seattle, WA 98195, - - 2010
Duchenne muscular dystrophy (DMD) is a progressive and fatal genetic disorder of muscle degeneration. Patients with DMD lack expression of the protein dystrophin as a result of mutations in the X-linked dystrophin gene. The loss of dystrophin leads to severe skeletal muscle pathologies as well as cardiomyopathy, which manifests as ...
Maron Barry J - - 2010
Lysosome-associated membrane protein (LAMP2) cardiomyopathy is an X-linked and highly progressive myocardial storage disorder associated with diminished survival, which clinically resembles sarcomeric hypertrophic cardiomyopathy. As shown here in a young woman, the natural history of LAMP2 may demonstrate an extreme profile of left ventricular remodeling with regression of hypertrophy (i.e. ...
Pokorney Sean D - - 2010
Patients with obstructive hypertrophic cardiomyopathy who undergo septal myectomy are at risk for developing postoperative atrial fibrillation. Amiodarone is effective in treating this arrhythmia but is associated with multiple adverse effects, often with delayed onset. A novel case is described of a patient who developed type 2 amiodarone-induced hyperthyroidism that ...
Berger Stuart - - 2010
There are various underlying causes of tachycardia-induced cardiomyopathy (TIC), and it is critical that it be considered in any patient who presents with a newly diagnosed dilated cardiomyopathy. Unlike most other forms of cardiomyopathy, TIC should be considered a treatable form of cardiomyopathy and it is imperative that the diagnosis ...
Cacciotti Luca - - 2010
Stress cardiomyopathy is a reversible left ventricular dysfunction triggered by emotional stress. We describe a variant of transient left ventricular ballooning in a patient in which basal and midventricular segments are affected. This confirms that there is not just one ventricular dysfunction pattern in Takotsubo cardiomyopathy. The involvement of only ...
Ashikhmina Elena A - - 2011
We sought to summarize our recent experience with intraoperative monitoring for management of patients undergoing surgical myectomy for hypertrophic obstructive cardiomyopathy with emphasis on dynamic left ventricular outflow tract obstruction. We also analyzed the impact of these data on surgical decision-making and adequacy of septal myectomy. We retrospectively analyzed the ...
Otsuka Koji - - 2010
The ubiquitin (Ub)-proteasome system (UPS) is an important proteolytic mechanism for selecting and digesting cytotoxic proteins. The aim of this study is to elucidate expression and in situ localization of the UPS in the myocardium from patients with dilated cardiomyopathy (DCM) with refractory heart failure. The expression profile of the ...
Yuan Jiansong - - 2010
To evaluate myocardial infarction and describe the early to mid-term changes induced by percutaneous ventricular septal ablation (PVSA) in symptomatic patients with hypertrophic cardiomyopathy using cardiac magnetic resonance imaging. Cardiac magnetic resonance imaging was performed before and 1 week and 1 year after PVSA in 52 patients. The relation between ...
Peña James R JR Department of Medicine, Section of Cardiology, University of Illinois at Chicago, Chicago, IL 60612, - - 2010
Familial hypertrophic cardiomyopathy (FHC) is an autosomal dominant genetic disorder linked to numerous mutations in the sarcomeric proteins. The clinical presentation of FHC is highly variable, but it is a major cause of sudden cardiac death in young adults with no specific treatments. We tested the hypothesis that early intervention ...
Brown Morgan L - - 2011
In natural history studies, increased left ventricular mass and wall thickness are strongly associated with increased late mortality in patients with hypertrophic cardiomyopathy. Our objective was to determine the impact of left ventricular mass and wall thickness on survival after myectomy for hypertrophic obstructive cardiomyopathy. We reviewed the case histories ...
Beach Scott R - - 2010
Takotsubo cardiomyopathy is a novel acute cardiac syndrome characterized by transient regional systolic dysfunction of the left-ventricular apex and mid-ventricle, with hyperkinesis of the basal left ventricular segments, which has been associated with severe emotional or physical stress. This is the second published case report of takotsubo cardiomyopathy occurring in ...
Kawasaki Tatsuya - - 2011
A necropsy study of patients with hypertrophic cardiomyopathy (HCM) who died at a young age exhibited marked disarray and fibrosis in the mid-wall layer of the left ventricular (LV) myocardium. We assessed ultrasonic tissue characteristics in the three layers of the ventricular septum (VS), and correlated the result with long-term ...
Sirin B H - - 2010
We present a case of a 16-year-old girl who underwent infundibular myectomy for right ventricular outflow tract obstruction complicated by biventricular non-compaction. The pathogenesis of this condition remains unknown. In pediatric patients non-compaction cardiomyopathy is associated with other cardiac abnormalities and carries a high mortality as a result of heart ...
Cho Hyun-Ji - - 2010
Takotsubo cardiomyopathy is characterized by clinical features similar to those of acute myocardial ischemia, but without angiographic evidence of obstructive coronary artery disease. We present a patient with takotsubo cardiomyopathy following acute infarction involving the left insular cortex. A 52-year-old man was admitted with acute infarction of the left middle ...
Wess G - - 2010
Diastolic dysfunction occurs in many cats with hypertrophic cardiomyopathy (HCM). Less is known about systolic function in various stages of HCM. Myocardial strain analysis by tissue Doppler imaging (TDI) is a noninvasive echocardiographic method to assess systolic function that has not been reported previously in cats. To evaluate systolic function ...
Toko Haruhiro H Department of Cardiovascular Science and Medicine, Chiba University Graduate School of Medicine, Chiba, - - 2010
Dilated cardiomyopathy (DCM), characterized by dilatation and dysfunction of the left ventricle, is an important cause of heart failure. Many mutations in various genes, including cytoskeletal protein genes and contractile protein genes, have been identified in DCM patients, but the mechanisms of how such mutations lead to DCM remain unknown. ...
Pepe Martino - - 2011
Takotsubo cardiomyopathy is a left ventricle cardiomyopathy characterized by a reversible dyskinesia responsible for the typical apical ballooning aspect. The disease is considered benignant with a full recovery within a few weeks. We present the case of a 52-year-old woman who presented with angina diagnosed with Takotsubo cardiomyopathy on the ...
Ho Carolyn Y CY Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115, USA. - - 2010
Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy and a proposed substrate for arrhythmias and heart failure. In animal models, profibrotic genetic pathways are activated early, before hypertrophic remodeling. Data showing early profibrotic responses to sarcomere-gene mutations in patients with hypertrophic cardiomyopathy are lacking. We used echocardiography, cardiac magnetic resonance ...
Finsterer Josef - - 2010
Cardiac involvement (CI) in congenital muscular dystrophies (CMDs) has been only rarely investigated so far. By means of a systematic literature search we reviewed the literature about CI in CMD and found that CI is apparently absent in Ullrich CMD or CMD with integrin deficiency and only mild in Bethlem ...
Mansencal Nicolas - - 2010
Tako-Tsubo cardiomyopathy is a clinical entity mimicking acute coronary syndrome. Assessment of left ventricular function may be difficult using conventional echocardiography. to characterize left ventricular systolic function using contrast echocardiography in Tako-Tsubo cardiomyopathy. We prospectively studied 63 consecutive women admitted for suspected acute coronary syndrome who underwent coronary arteriography, biplane ...
Kulić Mehmed - - 2010
Hypertrophic obstructive cardiomyopathy (HOCM) is a primary, usually familial and genetically fixed myocardial hypertrophy, with dynamic left ventricular outflow tract obstruction. An alternative to surgical myectomy in the treatment of severe, drug refractory, HOCM is percutaneous transluminal septal myocardial ablation (PTSMA). We report a case of 24 year old female ...
Abe Takashi - - 2010
Peripartum cardiomyopathy (PPCM) is a form of heart failure that occurs in women within 1 month before delivery and 5 months after delivery. The outcome of PPCM is variable but improves significantly when appropriate medication is administered in the acute phase; furthermore, the outcome does not worsen even after discontinuation ...
Jurko Alexander A - - 2010
We present a case of an 18-year-old boy with neurofibromatosis type 1 and hypertrophic cardiomyopathy with systolic anteward movement of the anterior leaflet of the mitral valve. Gradient in the left ventricular outflow was 85 mm Hg secondary to subvalvular aortic stenosis with left ventricular diastolic dysfunction. The possibility of ...
Fernández-Pérez Gabriel C - - 2010
The objective of this article is to show how MRI findings can be used to differentiate Takotsubo cardiomyopathy from acute coronary syndrome. Takotsubo cardiomyopathy is a disorder that mimics acute coronary syndrome. MRI can show not only edema in the ventricular wall, which is diffuse and without arterial territory distribution, ...
Betrián Blasco Pedro - - 2010
A 29-week-old male foetus was diagnosed by foetal echocardiography with severe hypertrophic cardiomyopathy with systolic dysfunction and generalized oedema, undergoing a Caesarean section at 33 weeks. Mechanical ventilation and milrinone infusion were required during the first week. Systolic function and output parameters improved progressively. Metabolic and infectious screenings were negative. ...
Yang Hua - - 2010
Hypertrophic cardiomyopathy (HCM) is associated with considerable phenotypic heterogeneity. Previous studies have shown a relationship between the degree and location of hypertrophy and the prognosis of patients. The aim of this study was to compare left ventricular (LV) circumferential and longitudinal regional mechanics in patients with septal HCM and apical ...
Roberts William Clifford - - 2010
Four patients are described with either parchment-like thinning or partial but extensive myocyte depletion with severe fatty or fibrofatty infiltration of the free wall of the right ventricle in its outflow tract, including 2 previously reported patients who also had focal parchment-like thinning of the left ventricular free wall. Three ...
Hernandez Lazaro E - - 2010
We describe an atypical presentation of stress-induced cardiomyopathy - Takotsubo cardiomyopathy - in a 16-month-old boy previously diagnosed with cyclic vomiting and episodic hypertension. He developed features of cardiac failure and his echocardiogram showed left ventricular wall motion abnormality accompanied with elevated cardiac enzymes. Cardiac catheterisation showed no coronary arterial ...
Hoskins Anita C AC Cardiovascular Division, King's College London British Heart Foundation Centre, London, - - 2010
Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy, increased ventricular stiffness and impaired diastolic filling. We investigated to what extent myocardial functional defects can be explained by alterations in the passive and active properties of human cardiac myofibrils. Skinned ventricular myocytes were prepared from patients with obstructive HCM (two ...
Murphy Colm J - - 2010
The prevalence of primary (hereditary) hemochromatosis and secondary iron overload (hemosiderosis) is reaching epidemic levels worldwide. Iron-overload leads to excessive iron deposition in a wide variety of tissues, including the heart and endocrine tissues. Iron-overload cardiomyopathy is the primary determinant of survival in patients with secondary iron overload, while also ...
Shah N R - - 2010
Takotsubo cardiomyopathy (TC) is a condition which was first acknowledged in Japan and is characterized by a reversible systolic dysfunction of the apical or mid segments of the left ventricle. Typically affecting women in the post-menopausal population, it is triggered by intense emotional, physical or medical stress. Also known as ...
Nakae Ichiro - - 2010
Myocardial lipid overstorage may produce cardiomyopathy, leading to dysfunction, but advanced heart failure may cause lipolysis via sympathetic nerve activation. In the failing heart, the creatine kinase system may also be impaired. The aims of this study were to assess myocardial triglyceride (TG) and creatine (CR) in different types of ...
Olivotti Luca - - 2010
Stress cardiomyopathy is a newly described reversible cardiomyopathy, characterized by transient cardiac dysfunction usually precipitated by intense emotional or physical stress. Apart from the classical apical ballooning syndrome (Takotsubo), it is now increasingly recognized that the spectrum of stress cardiomyopathies is quite wide, with significant individual variations in clinical and ...
Patrianakos Alexandros P - - 2010
We describe two relatively asymptomatic cases diagnosed with the newly recognized unclassified cardiomyopathy, isolated left ventricular apical hypoplasia. The disease has been described mainly in cardiac magnetic resonance, whereas this study presents the echocardiographic characteristics of this new cardiomyopathy.
Tsipis Angelos - - 2010
The etiology of heart failure in dilated cardiomyopathy involves multiple agents. The purpose of this study was to investigate the presence of apoptosis-related proteins p53, bcl-2, and the defects of force transmission in end-stage dilated cardiomyopathy. We studied myocardial samples from 20 hearts with histologic findings of dilated cardiomyopathy. Myocardial ...
Psaltis Peter J - - 2011
Cardiac fibrosis plays an important prognostic role in nonischemic cardiomyopathy (NICM), making it a potential therapeutic target. Although electromechanical mapping has been used to identify myocardial scar and facilitate intramyocardial intervention in the setting of ischemic heart disease, its application has not been described in NICM. We assessed the detection ...
Stöllberger Claudia - - 2010
Takotsubo cardiomyopathy is characterized by transient left ventricular dysfunction, electrocardiographic changes, and minimal release of myocardial enzymes that mimic acute myocardial infarction in patients without coronary artery disease. Takotsubo cardiomyopathy is frequently triggered by emotional or physical stress and occurs primarily in post-menopausal women. The pathomechanism of Takotsubo cardiomyopathy is, ...
O'Hanlon Rory - - 2010
OBJECTIVES: We investigated the significance of fibrosis detected by late gadolinium enhancement cardiovascular magnetic resonance for the prediction of major clinical events in hypertrophic cardiomyopathy (HCM). BACKGROUND: The role of myocardial fibrosis in the prediction of sudden death and heart failure in HCM is unclear with a lack of prospective ...
Maron Martin S - - 2010
Cardiovascular magnetic resonance (CMR) is a high spatial resolution, 3-dimensional tomographic imaging technique which may identify regions of massive left ventricular hypertrophy (particularly when confined to the anterolateral free wall) in which the extent of wall thickness is underestimated with traditional 2-dimensional echocardiography in patients with hypertrophic cardiomyopathy (HC). This ...
Tanriver Y - - 2010
The clinical manifestation of pheochromocytomas is highly variable and can closely resemble numerous clinical conditions. Here, we report on two cases of patients with pheochromocytoma, which manifested as sepsis or cardiomyopathy. The first patient initially presented with bacterial urosepsis due to klebsiella oxytoca. Despite effective antibiotic therapy, the patient developed ...
Berman Marius - - 2010
Most deaths in the first 30 days after cardiac transplantation are due to failure of the donor heart, often with the clinical picture of right ventricular failure. Indeed, there is a significant reduction in contractility of the human donor heart and loss of contractile reserve before and soon after transplantation. ...
Freel K M - - 2010
Postmortem and histological examination of the hearts from two horses, a five-year-old Clydesdale gelding and a 15-year-old cob gelding, revealed changes characteristic of arrhythmogenic right ventricular cardiomyopathy. In both cases, on gross examination, the right ventricular endocardium and interventricular septum were almost entirely replaced with a gelatinous yellow fibroareolar tissue. ...
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