To date, only weak associations have been reported between idiopathic dilated cardiomyopathy (DCM) and certain HLA class II alleles. Associations between HLA class II alleles and specific causes of DCM, especially myocarditis, have as yet not been systematically investigated. Typing of HLA DQB1* allele was performed using a sequence-specific primer-polymerase ...

A 66-year-old man with a diagnosis of monoclonal gammopathy of unknown significance was referred for investigation of bilateral transudative pleural effusions by the cardiology team. Echocardiography, myocardial perfusion scanning and left heart catheterisation were all normal or non diagnostic. Given significant occupational asbestos exposure in his twenties he underwent thoracoscopic ...

Cardiac complications from brucellosis are unusual and usually manifest as endocarditis. The other possible complication is myocardial involvement. Brucella myocarditis and development of heart failure is a very rare complication of brucellosis. We present a patient with new onset heart failure due to brucella myocarditis treated with favorable antibiotic therapy.

Eosinophilic myocarditis is characterized by progressive myocardial damage that results in heart failure and death. Herein, we present the case of a 54-year-old man who presented with symptoms of acute myocardial infarction. Normal coronary angiographic results and the presence of elevated levels of peripheral-blood eosinophilia prompted an endomyocardial biopsy that ...

Myasthenia gravis is an autoimmune neuromuscular disorder characterized by skeletal muscle involvement, causing muscle weakness and fatigue. The prevalence of the disease is approximately 1:7500 with a maximal prevalence during the second and third decade in women and the fifth and sixth decade in men, although it may appear at ...

A 75-year-old man with cardiac amyloidosis was presumptively diagnosed as having cardiac syndrome X. Early clinical presentation was repeated episodes of angina pectoris without angiographically significant stenosis of the coronary arteries, so the diagnosis of cardiac syndrome X was initially given. However, his anginal symptoms worsened despite appropriate medication. He ...

A 19-year-old man was transferred to hospital because of myocarditis with cardiogenic shock. Echocardiography showed a left ventricular ejection fraction of 23.8% and an intermediate amount of pericardial effusion. The patient immediately received an intra-aortic balloon pump and percutaneous cardiopulmonary support. Right ventricular endomyocardial biopsy was performed in the acute ...

Although the presence of abnormal late gadolinium enhancement (LGE) in cardiac amyloidosis has been well established, its prognostic implication and utility to identify cardiac involvement in patients with systemic amyloidosis is unknown. The aim of this study was to assess the diagnostic and prognostic significance of cardiovascular magnetic resonance imaging ...

Fibrosis is an established morphological feature of the structural myocardial remodeling that occurs in several cardiac diseases. This feature confers an increased risk for adverse cardiovascular events such as ventricular dysfunction and arrhythmias. The molecular mechanisms that lead to a fibrogenic cardiac phenotype are still being elucidated. A small number ...

Inflammatory cardiomyopathy is a common cause of heart failure developing on a basis of cardiac inflammation. Cardiac inflammation - or myocarditis - is usually triggered by infections or cardiac damage of any cause. Experimental autoimmune myocarditis refers to a CD4(+) T cell-mediated mouse model of inflammatory cardiomyopathy. So far, the ...

We report herein the coronary flow (CF) pattern determined by transthoracic Doppler echocardiography (TTDE) in two children with biventricular endomyocardial fibrosis (EMF). Endomyocardial biopsy showed various degrees of cardiac fibrosis in both patients as well as signs of chronic inflammation in one of the patients. TTDE showed a significant increase ...

Cardiac MRI is known to be diagnostically valuable in cardiac amyloidosis. Several features are frequently found, including myocardial hypertrophy, diastolic dysfunction, a faster gadolinium blood wash out, pleural and pericardial effusions, and diffuse myocardial delayed enhancement. Cardiac MR facilitates the detection of cardiac amyloidosis and allows longitudinal assessment of myocardial ...

Here we describe an unusual case of giant cell myocarditis (GCM) found in the left atrial appendage. Giant cell myocarditis is a rare entity in itself, while isolated left atrial GCM has only been reported on a few occasions. We describe a patient who underwent mitral valve replacement for rheumatic ...

Cardiac fibrosis is a major pathogenic factor in a variety of cardiovascular diseases and refers to an excessive deposition of extracellular matrix components in the heart, which leads to cardiac dysfunction and eventually overt heart failure. Evidence is accumulating for a crucial role of connective tissue growth factor (CTGF) in ...

INTRODUCTION: Case studies indicate that cardiac sarcoid may mimic the clinical presentation of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); however, the incidence and clinical predictors to diagnose cardiac sarcoid in patients who meet International Task Force criteria for ARVD/C are unknown. METHODS AND RESULTS: Patients referred for evaluation of left bundle ...

OBJECTIVES: The purpose of this study was to investigate a noninvasive method for quantifying diffuse myocardial fibrosis with cardiac magnetic resonance imaging (CMRI). BACKGROUND: Diffuse myocardial fibrosis is a fundamental process in pathologic remodeling in cardiomyopathy and is postulated to cause increased cardiac stiffness and poor clinical outcomes. Although regional ...

We briefly review the disease processes for four young healthy soldiers who presented to our emergency department with serious cardiac pathological conditions. We present two unusual cases of myocardial infarction, a coronary artery aneurysm, and a case of smallpox vaccine-induced myocarditis/pericarditis. Our intent is to encourage others in military medicine ...

We describe magnetic resonance (MR) aspect of cardiac glycogenesis in a 49-years old man, presented a progressively declining cardiac function and negative coronary angiography. Delayed enhancement MR confirmed non-ischemic pattern with unusual diffuse distribution of Gadolinium. Cardiac biopsy revealed a Glycogen Storage Disease, extralysosomial type. Cardiac MR with analysis of ...

Acute cardiac allograft rejection (ACAR) has been associated with a poor prognosis. The early diagnosis of ACAR necessitates the accurate detection of myocyte damage. Nuclear damage activates p53, a transcription factor that initiates apoptosis and repair. Endomyocardial biopsies (n=25) from 10 cardiac allograft recipients were stained for nuclear p53. The ...

This report details an unusual case of Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorder (PTLD) involving the epicardial coronary arteries and myocardial arterioles of an allograft heart, morphologically mimicking vasculitis, a finding associated with acute rejection, cardiac allograft vasculopathy (CAV), infection and infarction. Recognition of this novel PTLD pattern as a ...

We report the first observation of diffuse subendocardial and myocardial delayed enhancement on cardiac MRI in a 50-year-old patient with recurrent multiple myeloma but without evidence of amyloidosis. She presented with advanced heart failure and severe restrictive cardiomyopathy. Myocardial biopsy revealed endomyocardial fibrosis. The case was associated with development of ...

An 11-year-old boy with serologically confirmed Chlamydophila pneumoniae infection presented with clinical, laboratory, and echocardiographic changes consistent with myopericarditis. No reports on C. pneumoniae myopericarditis in children are found in the medical literature. The boy, previously healthy, presented with fever, rash, constitutional symptoms, elevated acute phase reactants, elevated cardiac enzymes, ...

BACKGROUND: Cardiac amyloidosis is characterized by amyloid infiltration resulting in extracellular matrix disruption. Amyloid cardiomyopathy due to immunoglobulin light chain protein (AL-CMP) deposition has an accelerated clinical course and a worse prognosis compared with non-light chain cardiac amyloidoses (ie, forms associated with wild-type or mutated transthyretin [TTR]). We therefore tested ...

INTRODUCTION: Idiopathic dilated cardiomyopathy (DCM) is, together with ischemic heart disease, the major cause of end-stage heart failure leading to heart transplantation. However, an unknown percentage of patients with this diagnosis has inflammatory foci found in the histopathological study of the explanted heart. This fact suggests an undetected process of ...

Sarcoidosis is a multisystem disorder of unknown cause, characterized pathologically by noncaseating granulomas. It is more commonly seen in younger adults and African Americans. Myocardial involvement occurs in at least 25% of patients with sarcoidosis and is associated with poor prognosis. In spite of recent advances in imaging modalities, early ...

Amyloidosis has been defined as an infiltrative disorder primarily caused by extracellular tissue deposition of amyloid fibrils. There are at least twenty five different human and eight different animal proteins precursors of amyloid fibrils. Subtypes are differentiated by means of immunohistochemical and genetic testing, with prognosis and therapeutic strategies different ...

AIMS: The apical ballooning syndrome (ABS) is a new diagnostic entity which is increasingly recognized. Precise magnetic resonance imaging (MRI) data are not yet available and there is little evidence for the differential diagnosis of ABS assessed by MRI. METHODS AND RESULTS: Between January 2005 and January 2008, 6100 consecutive ...

Endomyocardial fibrosis (EMF) is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions affecting the inflow tract and apex of one or both ventricles, which show fibrous thickening of the endocardium and adjacent myocardium. Surgical treatment is recommended for patients in functional classes III or IV (New York Heart ...

We report a case of myocarditis mimicking acute lateral myocardial infarction and treated as such initially, which was complicated by ventricular fibrillation a few hours after admission to the intensive care unit. The correct diagnosis was rapidly made using a low-dose delayed-enhanced cardiac multidetector computed tomography scan performed immediately after ...

Myocyte diameter, fractional area of collagen, intensity of myocarditis and parasite persistence (explored by immunohistochemistry and PCR) were evaluated in serial sections of endomyocardial biopsies from 29 outpatients with chronic chagasic cardiopathy. The patients, 25 males and four females with a mean (S.D.) age of 43 (9) years, were subsequently ...

Endomyocardial fibrosis is a rare condition that occurs primarily in tropical countries. It can often mimick a variety of other common cardiac conditions such as apical hypertrophic cardiomyopathy and Ebstein anomaly. We report a case of a left ventricular mass that at histologic examination was found to be endomyocardial fibrosis.

Heart transplantation (HTx) is a well-established treatment for severe cardiac failure. However, HTx for cardiac sarcoidosis is rare; less than 80 patients have been reported worldwide. In many patients, the diagnosis was not made prior to HTx. The aim of this study was to describe the use of HTx in ...

Acute fulminant myocarditis may present with cardiogenic shock refractory to inotropics and intra-aortic balloon pumping (IABP). Benefit of extracorporeal membrane oxygenation (ECMO) support has been established. The effectiveness of combination with ECMO or IABP and activated protein C (drotrecogin alpha; Xigris) in treatment has yet to be defined. Four patients ...

Radix Astragali, a Chinese medicinal herb, consists of polysaccharides and flavonoids as its main active ingredients. It has been widely used for treatment of cardiovascular diseases such as heart failure, angina pectoris, myocardial infarction and stroke in Asian countries. This study was designed to evaluate the effect of Radix Astragali ...

A 42 year-old female carrier of Duchenne muscular dystrophy (DMD) was referred with suspected subacute myocarditis and non-sustained ventricular tachycardia. Echochardiography and cardiac catheterization revealed severely reduced left ventricular function (LVF). Coronary artery disease was excluded. Cardiac magnetic resonance imaging showed transmural, intramural and subepicardial late gadolinium enhancement. Myocardial biopsy ...

Dengue fever is manifested by a sudden onset of fever (http://en.wikipedia.org/wiki/Fever), with severe headache, myalgias (http://en.wikipedia.org/wiki/Myalgia), arthralgias (http://en.wikipedia.org/wiki/Arthralgia) and characteristic bright red petechia (http://en.wikipedia.org/wiki/Petechia). The exact incidence and pathophysiological mechanism of dengue myocarditis remain obscure, but most of these cases are self-limiting. Fatal dengue myocarditis is a very rare complication ...

BACKGROUND: The objective of this study was to identify the prognostic indicators in patients with suspected myocarditis who underwent endomyocardial biopsy. METHODS AND RESULTS: Between 1994 and 2007, 181 consecutive patients (age, 42+/-15 years) with clinically suspected viral myocarditis were enrolled and followed up for a mean of 59+/-42 months. ...

A 71-year-old woman was admitted to our department because of acute myocarditis. She was ameliorated with conventional heart failure treatment, however she developed left ventricular dilatation and cardiac troponin T (cTnT) was elevated again to >1.0 ng/ml 6 month after the first admission. She was re-admitted because of recurrent decompensated ...

Necrotizing fasciitis (NF) is an infection localized at the fascial structures (both the superficial and deep ones) layering the muscles though never affecting them. NF death rate is very high (20-40%). NF can be a post-surgery, traumatic or infective complication and its prognosis quoad vitam is fatal without a timely ...

An apparently healthy 43-year-old man was submitted to cardiological evaluation for sport activity. Two-dimensional echocardiography led to suspicion of noncompaction deformity, later confirmed by magnetic resonance imaging (MRI), left ventricular catheterization and endomyocardial biopsies. To exclude life-threatening arrhythmias, the patient was submitted to an electrophysiological study and to a real-time ...

Early detection of myocardial dysfunction in Fabry disease (FD) cardiomyopathy suggests the contribution of myofilament structural alterations. Six males with untreated FD cardiomyopathy submitted to cardiac studies, including tissue Doppler imaging and left ventricular endomyocardial biopsy. Active and resting tensions before and after treatment with protein kinase A (PKA) were ...

Cardiac sarcoidosis can have a variety of manifestations including conduction disease, congestive heart failure, valvular heart disease, pericardial effusions, tamponade, ventricular arrhythmias and sudden cardiac death. In patients with sarcoidosis, the reported incidence of cardiac involvement ranges from 20% in US autopsy studies to nearly 60% in Japan, where it ...

BACKGROUND: A 37-year-old male with a history of palpitations and ventricular tachycardia was diagnosed with arrhythmogenic right ventricular dysplasia/cardiomyopathy on the basis of clinical assessment, electrocardiography and echocardiography. Over the following 3 years the patient progressed to end-stage heart failure and eventually underwent heart transplantation. Histological analysis of the explanted ...

OBJECTIVES: We sought to evaluate the diagnostic performance of cardiovascular magnetic resonance imaging (CMRI) for detection of cardiac amyloidosis compared with endomyocardial biopsy (EMB) in a clinical routine setting. BACKGROUND: For the clinical workup of heart failure with restrictive filling, pattern cardiac amyloidosis is an important differential diagnosis that is ...

BACKGROUND: Hypothyroid state may induce cardiac muscle impairment such as diastolic dysfunction and abnormal relaxation time. Advanced heart failure in hypothyroid patients has been described only in severe symptomatic cases, mostly during myxedematous coma. METHODS AND RESULTS: We describe an unusual case of asymptomatic patient with hypothyroidism who presented with ...

Endomyocardial fibrosis (EMF) is a neglected tropical cardiomyopathy of unknown etiology and pathogenesis that is common in certain tropical areas of Africa, Asia, and South America. It affects predominantly children and young adults. Endocardial fibrosis is the hallmark of this restrictive cardiomyopathy leading to restriction to diastolic filling of the ...

We report an adolescent with giant cell myocarditis (GCM) mimicking tachycardia-induced cardiomyopathy. His electrocardiogram (ECG) was typical for an incessant form of fascicular ventricular tachycardia. The patient rapidly deteriorated and required support using extracorporeal membrane oxygenation (ECMO). Biopsy revealed GCM with massive myocyte necrosis. He was successfully heart transplanted 6 ...

Dilated cardiomyopathy (DCM), a leading cause of heart failure and heart transplantation in younger adults, is characterized by dilatation and impaired contraction of the left or both ventricles; it may be idiopathic, familial/genetic (20-30%), viral, and/or immune. On endomyocardial biopsy there is chronic inflammation in 30-40% of cases. Mutations in ...

OBJECTIVE: To evaluate the incidence of coronary vasospasm as a possible pathophysiological mechanism causing chest pain symptoms in patients with clinically suspected myocarditis. DESIGN AND SETTING: Prospective study in a teaching hospital. PATIENTS: 85 patients who presented at hospital with atypical chest pain and demonstrated clinical signs suggestive of myocarditis. ...

BACKGROUND: A previously fit and healthy 30-year-old man reported experiencing palpitations accompanied by nausea, sweating and presyncope. These symptoms were found to be associated with episodes of nonsustained ventricular tachycardia. He was a nonsmoker, did not drink excessively, denied illicit drug use and had no family history of structural cardiac ...