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Results 201 - 250 of 1707
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Maron Martin S - - 2009
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy with substantial heterogeneity in phenotypic expression and clinical course. Traditionally, two-dimensional echocardiography has been the easiest and most reliable technique for establishing a diagnosis of HCM. However, cardiovascular magnetic resonance (CMR) has emerged as a novel, three-dimensional tomographic imaging technique, which ...
Cecchi Franco - - 2009
Microvascular dysfunction can be demonstrated in most patients with hypertrophic cardiomyopathy (HCM), both in the hypertrophied and nonhypertrophied myocardial walls, mostly due to intimal and medial hyperplasia of the intramural coronary arteries and subsequent lumen reduction. As a consequence, regional myocardial ischemia may be triggered by exercise, increased heart rate, ...
Vasquez Andres F - - 2009
A 55-year-old man evaluated for syncope had mild and gradually progressive left ventricular systolic dysfunction on initial workup. Although not identified initially, repeat cardiac magnetic resonance one year later demonstrated the presence of trabeculations in the left ventricle consistent with the diagnosis of isolated non-compaction of the left ventricular myocardium. ...
Yajima Noriyuki - - 2009
Heart failure is important in determining the prognosis of cardiomyopathy caused by mitochondrial gene abnormalities. We report herein the case of a patient with pericardial effusion and heart failure in whom mitochondrial cardiomyopathy was definitively diagnosed. A 56-year-old woman consulted her primary physician with exertional dyspnea. Examination revealed edema and ...
Stern Adam W - - 2009
Hypertrophic cardiomyopathy (HCM) was diagnosed during postmortem examination of 2 captive adult Bennett's wallabies (Macropus rufogriseus rufogriseus). The wallabies were members of a mob (herd) of 3 wallabies, and 2 died spontaneously without clinical signs of heart failure being detected. Gross lesions in both cases included marked concentric hypertrophy of ...
van de Donk N W C J - - 2009
We report on a 73-year-old man with a toxic multinodular goitre, which was treated with radioiodine therapy (I-131) without pretreatment with an antithyroid drug. Four weeks later he presented with rapidly progressive dyspnoea and a significant increase in free thyroxin. The electrocardiogram showed ST -segment elevation, and echocardiography demonstrated apical ...
McCauley Mark D MD Department of Medicine, Baylor College of Medicine, Houston, TX 77030, - - 2009
Arrhythmogenic cardiomyopathies are a heterogeneous group of pathological conditions that give rise to myocardial dysfunction with an increased risk for atrial or ventricular arrhythmias. Inherited defects in cardiomyocyte proteins in the sarcomeric contractile apparatus, the cytoskeleton and desmosomal cell-cell contact junctions are becoming recognized increasingly as major causes of sudden ...
Udink ten Cate Floris E A - - 2010
BACKGROUND: Ventricular preexcitation may be associated with dilated cardiomyopathy, even in the absence of recurrent and incessant tachycardia. METHODS: This report describes the clinical and electrophysiologic characteristics of 10 consecutive children (6 males), with median age of 8 years (range, 1-17 years), who presented with dilated cardiomyopathy and overt ventricular ...
Olivotto Iacopo - - 2009
Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease, characterized by complex pathophysiology, heterogeneous morphology, and variable clinical manifestations over time. Besides cardiac hypertrophy, the HCM phenotype is characterized by a host of manifestations, including mitral valve and subvalvar abnormalities, subaortic and mid-ventricular left ventricular (LV) obstruction, microvascular dysfunction, ...
Okayama Satoshi - - 2010
A 72-year-old woman with hypertrophic obstructive cardiomyopathy underwent coronary computed tomography (CT) angiography that showed perfusion of the hypertrophied interventricular septum by the first septal artery. One month after percutaneous transluminal septal myocardial ablation, repeat CT perfusion imaging identified a transmural myocardial infarction in the basal hypertrophic interventricular septum which ...
Blanc P - - 2010
We report a case of a rare form of midventricular Tako Tsubo cardiomyopathy characterized by left ventricular angiography and cardiac MRI.
Fuster Valentin - - 2009
Evolving imaging modalities in hypertrophic cardiomyopathy (HCM), such as tissue Doppler, speckle tracking, measures of myocardial blood flow, and cardiac magnetic resonance with gadolinium enhancement, have advanced our understanding of the pathogenesis of myocardial dysfunction in hypertrophic cardiomyopathy. These modalities have the potential to differentiate HCM from other causes of ...
Gologorsky Edward - - 2010
The authors discuss and present transesophageal echocardiographic images of intraoperative tako-tsubo cardiomyopathy in a female patient under general anesthesia. Intraoperative transesophageal echocardiography was performed in an attempt to manage a sudden episode of hypotension and mild pulmonary edema coincidental with surgical incision. The suspected diagnosis of stress cardiomyopathy was confirmed ...
Ciftci Haci - - 2009
Hypertrophic cardiomyopathy is a genetic myocardial disorder. In such patients, myocardial bridging is the most frequent encountered coronary arterial anomaly. Patients may, however, on occasion, present with other much rarer malformations of the coronary arteries. Duplication of the right coronary artery is a very rare anomaly. We have now encountered ...
Braunwald Eugene - - 2009
Hypertrophic obstructive cardiomyopathy (HOCM) has four major features: (1) severe left ventricular hypertrophy, often most prominent in the basal interventricular septum; (2) frequent familial occurrence with autosomal dominant transmission; (3) occurrence of sudden cardiac death that is usually considered to be due to ventricular fibrillation; and (4) presence of hemodynamic ...
Hsu Po-Shun - - 2009
Hamartoma of mature cardiac myocytes is a form of cardiac tumor that shares some features with hypertrophic cardiomyopathy and rhabdomyomas. Here we describe a patient with a ventricular hamartoma complicated with ventricular tachycardia. Resection was not practical because of difficulty in maintaining the ventricular geometry, so heart transplantation was done.
Kurdi Maher E - - 2009
Dandy Walker malformation (DWM) is a rare congenital brain anomaly characterized by cystic dilation of the fourth ventricle and hypoplasia of the cerebellar vermis. Other extracranial anomalies can be associated, including cardiac defects. We report a rare patient with DWM associated with progressive heart failure secondary to hypertrophic cardiomyopathy. He ...
Kargin Ramazan - - 2009
We present a case of hypertrophic cardiomyopathy with anomalous insertion of mitral valve chordae tendineae diagnosed with two-dimensional echocardiogram. A high gradient was found in left ventricular outflow tract (LVOT) obstruction, which was attributed to the fixed gradient caused by anomalous insertion of chordae tendineae in addition to the gradient ...
Nozyński J - - 2009
BACKGROUND: Antiapoptotic as well as replacement and proliferative mechanisms take place in the myocardium in dilated cardiomyopathy (DCM) and ischemic heart disease (IHD). We sought to estimate antiapoptotic, proliferative and replacement activities in cardiomyopathies. MATERIALS: The study groups included seven hearts with DCM and eight with IHD, which had been ...
Ghio Stefano - - 2009
Hypertrophic cardiomyopathy (HCM) is a genetic disease histologically characterized by a profound disarray of myocardial fibres and by local fibrosis. We sought to characterize regional left ventricular contractility in HCM patients using deformation analysis and to compare it with the presence or absence of delayed enhancement in cardiac magnetic resonance ...
Assenza Gabriele Egidy - - 2009
The authors report the case of a patient with hypertrophic cardiomyopathy who developed progressive and severe left ventricular wall thinning, as assessed by two-dimensional echocardiography, despite a preserved supranormal ejection fraction and an absence of cardiac symptoms. Extensive fibrosis was identified on cardiovascular magnetic resonance.
Amano Yasuo - - 2009
To assess the myocardial damage in hypertrophic cardiomyopathy (HCM) using contrast-enhanced myocardial T1-weighted scout (Look-Locker) magnetic resonance imaging (MRI). Twenty-three patients with HCM and seven comparative patients without known HCM serving as controls underwent cine, contrast-enhanced myocardial T1-weighted scout and delayed-enhancement MRI using a 1.5T unit. Intervals of null points ...
Fan Katherine Y Y - - 2009
We report a patient with isolated left ventricular non-compaction diagnosed by echocardiography and cardiac magnetic resonance imaging. She developed refractory congestive heart failure and subsequently underwent successful heart transplantation. This type of cardiomyopathy is thought to be caused by the arrest of normal embryogenesis of the endocardium and myocardium. The ...
Fidziańska Anna - - 2010
Endomyocardial biopsy of a patient in transition stage from hypertrophic cardiomyopathy to heart failure was investigated. The tissue showed hypertrophy, atrophy of myocytes and an increased amount of fibrosis. In addition, numerous cardiomyocytes revealed ubiquitin positive inclusions. Ultrastructural analysis indicated that cardiomyocytes contained typical autophagic vacuoles including mitochondria, glycogen granules, ...
Labombarda Fabien - - 2009
OBJECTIVE: Left ventricular mechanical dyssynchrony has been poorly studied in the pediatric population with dilated cardiomyopathy. We investigated the degree of dyssynchrony in children with dilated cardiomyopathy using tissue Doppler imaging and speckle tracking strain. METHODS: Twenty-five children with dilated cardiomyopathy were compared with healthy subjects. Left ventricular mechanical dyssynchrony ...
Koulouris Spyridon - - 2009
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is one of the major causes of sudden death. Myocardial atrophy with subsequent fibro-fatty replacement predominantly affects right ventricular myocardium and results in global and regional dysfunction as well as areas of slow conduction and dispersion of refractoriness, which are prerequisites for reentrant ventricular tachyarrhythmias. ...
Rossi Ana P - - 2009
OBJECTIVE: To describe a case of recurrent takotsubo cardiomyopathy in a patient with pheochromocytoma. METHODS: We present a case report, including clinical and laboratory data. In addition, the current relevant literature pertaining to pheochromocytoma and takotsubo syndrome is reviewed and summarized. RESULTS: In 2004, an 81-year-old woman with no history ...
Chelliah Rajesh K - - 2009
Distinguishing physiological left ventricular hypertrophy of an athlete's heart from that of pathological left ventricular (hypertrophic cardiomyopathy) can be difficult despite the advent of new imaging techniques. Nevertheless, the final diagnosis is of utmost importance as it will have a profound impact on an individual's life. A diagnosis of hypertrophic ...
Kirvalidze I - - 2009
An article reviews etiopathogenesis of cardiomyopathy. Regardless of the fact that many early days' fundamental investigations are devoted to cardiomyopathy problem, still there is no common consideration concerning the etiology, pathogenesis, morphogenesis, classification and therapy of cardiomyopathy as well. Problem concerning the etiology of cardiomyopathy appear of particular interest. Great ...
Irqsusi Marc - - 2009
In patients with arrhythmogenic right ventricular dysplasia (ARVD), the right ventricular myocardium histologically discloses atrophy paralleled by fibrofatty or fatty replacement. Apoptosis is believed to be a putative major pathogenetic mechanism. Altogether, our knowledge of genetics, etiology and pathophysiology of ARVD has increased impressively in the last few years, and ...
Meng L - - 2009
Takotsubo cardiomyopathy, or stress-induced cardiomyopathy, is a clinical syndrome mimicking acute myocardial infarction. We report a case of a 55-year-old man who developed Takotsubo cardiomyopathy during emergence from general anaesthesia for an elective procedure to remove a spinal cord stimulator The perioperative state is known to induce stress, but there ...
Maron B J - - 2009
Sudden cardiac death in young competitive athletes is an important public health problem, although a relatively low-event-rate phenomenon. The single most common cardiovascular cause of these unexpected catastrophes is hypertrophic cardiomyopathy (HCM), accounting for about one-third of cases. Since the phenotypic expression of HCM is variable, and not uncommonly includes ...
Afonso Luis - - 2009
Myocardial clefts are congenital abnormalities related to myocardial fiber or fascicle disarray that have been described in healthy volunteers as well as in the setting of hypertrophic cardiomyopathy. A cleft or crypt can be described as a discrete, approximately "V" shaped fissure extending into but confined by the myocardium, with ...
Roura Santiago - - 2009
Idiopathic dilated cardiomyopathy (IDCM) is defined as myocardial dilatation and dysfunction in the absence of overt coronary heart disease. Myocardial injury and genetic or environmental factors can lead to the development of IDCM, which was historically characterized by marked abnormalities in the function and integrity of cardiomyocytes. However, cardiac endothelial ...
Pilichou Kalliopi - - 2009
Mutations in the cardiac desmosomal protein desmoglein-2 (DSG2) are associated with arrhythmogenic right ventricular cardiomyopathy (ARVC). We studied the explanted heart of a proband carrying the DSG2-N266S mutation as well as transgenic mice (Tg-NS) with cardiac overexpression of the mouse equivalent of this mutation, N271S-dsg2, with the aim of investigating ...
Roberts Jason D - - 2010
Arrhythmogenic right ventricular cardiomyopathy (ARVC) represents an inherited cardiomyopathy that manifests clinically with malignant ventricular arrhythmias, sudden cardiac death, and less commonly heart failure. The condition is characterized by replacement of the myocardium, primarily of the right ventricle, with fibrofatty tissue. Extensive fibrofatty replacement of the myocardium has been previously ...
Kleinfeldt Tilo - - 2009
Takotsubo cardiomyopathy (TTC) is a cardiac entity appreciated only recently mimicking acute myocardial infarction, often affects post-menopausal women and is triggered by preceding emotional or physical stress. Pathogenesis of TTC is unknown, recurrence of TTC in one individual and familial predisposition occurs. Expression profiling of cardiac genes in the acute ...
Daly Michael J - - 2010
Reversible stress-induced cardiomyopathy, i.e., Takotsubo cardiomyopathy, rarely presents in preoperative patients. We provide the case reports of two patients who presented with Takotsubo cardiomyopathy, which we surmise was due to excess endogenous catecholamine production in response to acute pain. Electrocardiogram revealed T-wave inversion, with peak Troponin-T elevation in each case, ...
Ho Yi-Lwun - - 2009
BACKGROUND: Mutations of cardiac troponin C (cTnC) can cause dilated cardiomyopathy in humans. METHODS AND RESULTS: Plasmids were constructed such that the reverse tetracycline-controlled transactivator (rtTA) was driven by the cardiac myosin light chain 2 promoter. This heart-specific rtTA bound another bidirectional promoter to express the green fluorescence protein reporter ...
Maron Barry J - - 2009
Dynamic obstruction to left ventricular (LV) outflow was recognized from the earliest (50 years ago) clinical descriptions of hypertrophic cardiomyopathy (HCM) and has proved to be a complex phenomenon unique in many respects, as well as arguably the most visible and well-known pathophysiologic component of this heterogeneous disease. Over the ...
Sherrid Mark V - - 2009
The shape of Doppler velocity tracings in obstructive hypertrophic cardiomyopathy offers insights into its pathophysiology. Inflection points are the points on a curve where its shape changes from concave to convex, or vice versa. These dynamic systolic abnormalities are caused: 1) by the amplifying nature of the obstruction; and 2) ...
Fefer Paul - - 2009
Takotsubo cardiomyopathy often presents to the cardiac catheterization laboratory masquerading as acute ST-elevation myocardial infarction (STEMI). Some of these patients present in shock secondary to dynamic left ventricular outflow tract (LVOT) obstruction. The typical patient is an elderly, hypertensive female with sigmoid deformity of the intraventricular septum. The management of ...
Wlodarska Elzbieta K - - 2010
Isolated ventricular noncompaction is considered to predominantly affect the left ventricle. It is characterized by increased left ventricular wall thickness and deep intertrabecular recesses with to-and-fro blood flow that remains in continuity with the ventricular flow. Aim of the study was to present a group of patients with isolated noncompaction ...
Vergez Magali - - 2009
Tako Tsubo cardiomyopathy has been described in a variety of stress situations, including several critical illness settings. We report the first case of this syndrome in a patient with multiple trauma. Tako Tsubo is an unusual cause of circulatory failure in such patients, to be distinguished from myocardial contusion and ...
Stöllberger Claudia - - 2011
Lamotrigine has the advantage to have an antidepressive effect and to be well tolerated in the majority of the cases. Lamotrigine, however, may exert cardiac side effects in patients with hypertrophic cardiomyopathy and latent obstruction of the left ventricular outflow tract, as illustrated by the following case report. In a ...
Villarroel Aicard H - - 2009
Takotsubo cardiomyopathy, also known as broken heart syndrome or stress cardiomyopathy, is a very interesting syndrome of acute transient left ventricular dysfunction, usually following significant emotional stress. It was first described in Japan nearly two decades ago and many aspects of its pathogenesis still remain poorly understood. The incidence of ...
Schwartzenberg Shmuel - - 2009
Isolated ventricular non-compaction is a frequently underdiagnosed rare congenital cardiomyopathy. The importance of diagnosing this cardiomyopathy lies especially in asymptomatic patients, screening relatives of index cases in order to focus on their follow-up, and searching for criteria warranting prophylactic anticoagulation, implantable cardioverter defibrillator and anti-remodeling drugs such as angiotensin-converting inhibitors. ...
Suvarna Jyoti C - - 2009
Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy postulated to be a defect in endomyocardial morphogenesis due to the embryonic arrest of the compaction of myocardial fibers. It could be isolated, without other structural heart defects; or associated with congenital heart defects. It is characterized by prominent ventricular myocardial ...
Di Bella Gianluca - - 2009
Lipomatous metaplasia is characterized by substitution of muscle fibers with adipose tissue in the context of a myocardial scar. Cardiac magnetic resonance is an accurate cardiac imaging technique with high tissue spatial resolution that can easily characterize scar tissue, adipose tissue and lipomatous metaplasia. We report a case of lipomatous ...
Cort??s R - - 2009
The nuclear lamins A/C play a critical role in maintaining the structure of the nuclear lamina and the organization of various proteins, such as emerin. These protein levels may play roles in the pathogenesis and clinical evolution of both ischemic (ICM) and dilated (DCM) cardiomyopathy. We evaluated the nuclear morphology ...
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