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By Eduardo Roque Cardiologista com foco em cardiologia hospitalar e cuidados intensivos. Professor de clínica médica.
Dóra Földeák, Attila Nemes, Anita Kalapos, Péter Domsik, Árpád Kormányos, László Krenács, Enikő Bagdi, Zita Borbényi
Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment...
November 2017: Orvosi Hetilap
Anit K Mankad, Keyur B Shah
PURPOSE OF REVIEW: Transthyretin (TTR)-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive, hypertrophic heart disease and may go undiagnosed. Transthyretin-derived amyloidosis accounts for 18% of all cases of cardiac amyloidosis. Thus, the study's purpose is to provide a comprehensive review of transthyretin cardiac amyloidosis. RECENT FINDINGS: Wild-type transthyretin (ATTRwt) protein causes cardiac amyloidosis sporadically, with 25 to 36% of the population older than 80 years of age are at risk to develop a slowly progressive, infiltrative amyloid cardiomyopathy secondary to ATTRwt...
August 24, 2017: Current Cardiology Reports
Mirela Tuzovic, Yukari Kobayashi, Matthew Wheeler, Christopher Barrett, Michaela Liedtke, Richard Lafayette, Stanley Schrier, Francois Haddad, Ronald Witteles
Cardiac involvement is common in patients with light-chain (AL) amyloidosis and portends a poor prognosis, although little is known about the changes in cardiac mechanics after chemotherapy. We sought to explore the relation between amyloidosis staging and baseline cardiac mechanics and to investigate short-term changes in cardiac mechanics after chemotherapy. We identified 41 consecutive patients from the Stanford Amyloid Center who had echocardiograms and free light-chain values before and after chemotherapy, along with 40 age- and gender-matched controls...
October 15, 2017: American Journal of Cardiology
Paolo Milani, Morie A Gertz, Giampaolo Merlini, Angela Dispenzieri
The aim of this survey was to describe the treatment decision making of expert physicians in when and how to treat patients with AL amyloidosis. Fifty amyloid expert physicians completed the survey. Autologous stem cell transplant (ASCT) was considered the first line therapy, if medically feasible, by 73% of the physicians. Excluding ASCT, cyclophosphamide-bortezomib-dexamethasone regimen was the preferred strategy by 72%. Depending on organ involvement, the goal for treatment was CR for 27-35% and very good partial response (VGPR) for 65-72%...
December 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
Eli Muchtar, Lori A Blauwet, Morie A Gertz
Restrictive cardiomyopathy (RCM) is characterized by nondilated left or right ventricle with diastolic dysfunction. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and prognosis. In this review, an overview of RCMs will be presented followed by a detailed discussion on 3 major causes of RCM, for which tailored interventions are available: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis...
September 15, 2017: Circulation Research
Abdullah M Alotaibi, Ahmed Aljizeeri, Mouaz Al-Mallah, Ahmed Alsaileek
We report a 26-year-old man who presented to the emergency department four times within a 4-year period with recurrent myocarditis. His presentations were characterized by chest pain, elevated troponin I, and normal coronary angiography. Endomyocardial biopsy showed nonspecific inflammatory process. Laboratory workup including viral screening and autoimmune markers were negative. Cardiac magnetic resonance imaging showed evidence of recurrent myocarditis with progressive appearance of new areas of myocardial delayed enhancement seen in each admission...
July 2017: Journal of the Saudi Heart Association
William Bracamonte-Baran, Daniela Čiháková
Myocarditis is the inflammation of the muscle tissues of the heart (myocardium). After a pathologic cardiac-specific inflammatory process, it may progress to chronic damage and dilated cardiomyopathy. The latter is characterized by systolic dysfunction, whose clinical correlate is heart failure. Nevertheless, other acute complications may arise as consequence of tissue damage and electrophysiologic disturbances. Different etiologies are involved in triggering myocarditis. In some cases, such as giant cell myocarditis or eosinophilic necrotizing myocarditis, it is an autoimmune process...
2017: Advances in Experimental Medicine and Biology
Giovanni Salvatore Camastra, Stefano Sbarbati, Massimiliano Danti, Luca Cacciotti, Raffaella Semeraro, Sabino Walter Della Sala, Gerardo Ansalone
AIM: To define the role of cardiac magnetic resonance (CMR) by analyzing a particular group of patients with suspected acute coronary syndrome (ACS) and normal coronary angiogram. METHODS: From January 2009 to December 2015, we examined 220 patients with clinical suspicion of ACS, Troponin elevation [the threshold used to define a positive Troponin T test (TnT) was 0.1 ng/mL] and no significant coronary disease at angiography (the patients were considered to have significant angiographic disease only a 50% stenosis was detected in any of their coronary arteries)...
June 28, 2017: World Journal of Radiology
Mateusz Kuffner, Agnieszka Pawlak, Maciej Przybylski
Viral infections of the heart cause serious clinical problems, either as infectious myocarditis, which usually is a consequence of acute infection or as idiopathic dilated cardiomyopathy, resulting rather from a chronic infection. This minireview presents an up-to-date view on pathomechanisms of viral infection of the heart tissues, the role of immune system in controlling infectious process at its various stages and current possibilities of recognizing viral infection of the heart with use of both cardiological and virological methods...
January 2, 2017: Polish Journal of Microbiology
M-Y Fu, Q-W Wang, Y Xue, F Xu, C-L Li, X-J An
OBJECTIVE: To analyze the correlation between chronic viral myocarditis (CVMC) in children, complicated with arrhythmia and thyroid hormone level. PATIENTS AND METHODS: 60 patients with CVMC complicated with arrhythmia were continuously selected (course of disease > 3 months) and they are were diagnosed with arrhythmia by the routine 18-lead electrocardiogram and 24-hour Holter; the average follow-up time is about 2 years, during which the left ventricular end-diastolic diameter (LVEDd), left ventricular ejection fraction (LVEF), the occurrence rate of malignant arrhythmia events, immune state of T cell and thyroid hormone level (FT3, FT4, TSH, TGAb and TPOAb) were compared...
July 2017: European Review for Medical and Pharmacological Sciences
Zijiao Ding, Mingzhen Yang, Yunyun Wang, Shifan Wu, Xingang Qiu, Qian Liu
Sudden cardiac death (SCD) is a major health challenge. The records of 769 autopsy cases of SCD examined at Tongji Medicolegal Expertise Center from January 2006 to December 2015 were retrospectively reviewed. The mean age of the cases was 46 years, excluding 27 victims in whom the exact age could not be confirmed. The highest incidence of SCD occurred among the 40- to 60-year-old group (45.0%). Male preponderance was observed in SCD cases (male: female ratio: 5.0:1), and this preponderance was even higher (8...
September 2017: Forensic Science, Medicine, and Pathology
Shuheng Wei, Jing Fu, Liang Chen, Shujing Yu
BACKGROUND Cardiac magnetic resonance imaging (CMRI) is considered to be useful for the diagnosis of myocarditis, and the Lake Louise Criteria (LLC) has been proved to be of significance as the standard of diagnosis. However, the diagnostic performance of LLC-based CMRI for myocarditis compared with endomyocardial biopsy (EMB) has not been quantitatively evaluated in a meta-analysis. MATERIAL AND METHODS The databases PubMed, Cochrane's Library, and EMBASE were searched to identify studies on LLC and its individual components for the diagnosis of myocarditis...
July 29, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
Behjat Javadi, Amirhossein Sahebkar
Myocarditis is an inflammatory disease of the myocardium associated with immune dysfunction which may frequently lead to the development of dilated cardiomyopathy. Experimental autoimmune myocarditis is an animal model which mimics myocarditis in order to allow assessment of the therapeutic effects of different molecules on this disease. We aimed to review the inflammatory and immunological mechanisms involved in the pathogenesis of the myocarditis and finding natural products and phytochemicals with anti-myocarditis activities based on studies of cardiac myosin-induced experimental autoimmune myocarditis in rodents...
July 27, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
Katie Murphy, Oral Waldo, Graham M Lohrmann, Henry D Tazelaar, Clinton E Jokerst, Farouk Mookadam
Reactive eosinophilia is associated with inflammatory bowel disease, but its association with eosinophilic myocarditis is rare. We report a case of a 42-year-old man who presented with hypovolemic shock secondary to diarrhea and recently diagnosed nonischemic cardiomyopathy (left ventricular ejection fraction, 0.29). Laboratory evaluation revealed marked peripheral eosinophilia. Cardiac magnetic resonance imaging showed evidence of subacute-to-chronic myocarditis, and endomyocardial biopsy results were consistent with eosinophilic myocarditis...
June 2017: Texas Heart Institute Journal
O Yu Karpova, R Yu Monovtsov, L I Dvoretsky, G R Cherepantsev, A V Magnitskiy
In this female patient eosinophilic myocarditis was complicated by myocardial rupture and hemopericardium with cardiac tamponade. Special features of this case were old age of the patient, fulminant course of the disease, myocardial infarction-like ECG changes, absence of peripheral blood eosinophilia. Presentation is accompanied by discussion of issues of epidemiology, clinical course, diagnostics, and possible causes of eosinophilic myocarditis.
May 2017: Kardiologiia
Claudia Seyler, Benjamin Meder, Tanja Weis, Thea Schwaneberg, Kerstin Weitmann, Wolfgang Hoffmann, Hugo A Katus, Andreas Dösch
AIMS: Non-ischemic cardiomyopathies (CMPs) comprise heart muscle disorders of different causes with high variability in disease phenotypes and clinical progression. The lack of national structures for the efficient recruitment, clinical and molecular classification, and follow-up of patients with non-ischemic CMPs limit the thorough analysis of disease mechanisms and the evaluation of novel diagnostic and therapeutic strategies. This paper describes a national, prospective, multicenter registry for patients with non-ischemic CMPs...
August 2017: ESC Heart Failure
Gustav Mattsson, Peter Magnusson
Electrical storm during the acute inflammatory phase caused by myocarditis may be resistant to antiarrhythmic therapy. Cardiac imaging including magnetic resonance tomography, positron emission tomography, and endomyocardial biopsy are crucial to guide potential therapeutic options. Optimal management involves a multidisciplinary approach, including expertise beyond cardiology.
August 2017: Clinical Case Reports
Josephine F Heidendael, Suzanne L Den Boer, Joanne G Wildenbeest, Michiel Dalinghaus, Bart Straver, Dasja Pajkrt
BACKGROUND: Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded...
January 2018: Cardiology in the Young
M Emrah Şelli, Anita C Thomas, David C Wraith, Andrew C Newby
Myocarditis, the principal cause of dilated cardiomyopathy and heart failure in young adults, is associated with autoimmunity to human cardiac α-myosin (hCAM) and the DR4 allele of human major histocompatibility II (MHCII). We developed an hCAM-induced myocarditis model in human HLA-DR4 transgenic mice that lack all mouse MHCII genes, demonstrating that immunization for 3weeks significantly increased splenic T-cell proliferative responses and titres of IgG1 and IgG2c antibodies, abolished weight gain, provoked cardiac inflammation and significantly impaired cardiac output and fractional shortening, by echocardiography, compared to adjuvant-injected mice...
June 2017: Journal of Molecular and Cellular Cardiology
Davide Muratori, Patrizia Pedrotti, Matteo Baroni, Armando Belloni, Giuseppina Quattrocchi, Angela Milazzo, Cristina Giannattasio, Alberto Roghi
Pheochromocytoma is a rare tumor, usually benign, potentially lethal in case of crisis with acute release of catecholamines. The heart is a target and the clinical presentation can mimic various cardiac conditions, thus rendering diagnosis elusive. Cardiac magnetic resonance is a valuable non-invasive diagnostic tool for the evaluation of cardiomyopathies; it allows the identification of catecholamine-induced myocarditis pattern and, in some cases, it can detect the primary tumor. The definitive treatment of pheochromocytoma is surgical, while the acute crisis may require mechanical support to circulation...
February 2017: Giornale Italiano di Cardiologia
2017-04-15 02:57:55
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