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restrictive cardiomyopathy

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https://www.readbyqxmd.com/read/28425041/familial-amyloid-cardiomyopathy-masquerading-as-chronic-guillain-barre-syndrome-things-are-not-always-what-they-seem
#1
Die Hu, Ling Liu, Shuguang Yuan, Yuhong Yi, Daoquan Peng
Familial amyloid cardiomyopathy is a challenging condition that mimics many other diseases, particularly in patients with pronounced neurological presentations and unexplained or equivocal cardiac abnormalities. In this case, a 57-year-old man was admitted for outpatient cardiological evaluation of progressive right heart failure and limb paraesthesias. The patient presented with hypertension, chronic Guillain-Barre syndrome, and sick sinus syndrome. Transthoracic echocardiograms showed a thickened ventricular wall and enlarged atrium...
April 19, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28421174/utility-of-cardiovascular-magnetic-resonance-derived-wave-intensity-analysis-as-a-marker-of-ventricular-function-in-children-with-heart-failure-and-normal-ejection-fraction
#2
Hopewell N Ntsinjana, Robin Chung, Paolo Ciliberti, Vivek Muthurangu, Silvia Schievano, Jan Marek, Kim H Parker, Andrew M Taylor, Giovanni Biglino
OBJECTIVE: This study sought to explore the diagnostic insight of cardiovascular magnetic resonance (CMR)-derived wave intensity analysis to better study systolic dysfunction in young patients with chronic diastolic dysfunction and preserved ejection fraction (EF), comparing it against other echocardiographic and CMR parameters. BACKGROUND: Evaluating systolic and diastolic dysfunctions in children is challenging, and a gold standard method is currently lacking...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28408455/cell-therapy-trials-in-congenital-heart-disease
#3
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28407059/septal-artery-diastolic-flow-profile-in-hypertrophic-restrictive-cardiomyopathy-doppler-square-root-sign
#4
Dhaval Desai, M Fuad Jan, Lindsey Kalvin, A Jamil Tajik
No abstract text is available yet for this article.
April 11, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28405937/new-cardiac-imaging-algorithms-to-diagnose-constrictive-pericarditis-versus-restrictive-cardiomyopathy
#5
REVIEW
Ahmad Mahmoud, Manish Bansal, Partho P Sengupta
PURPOSE OF REVIEW: Echocardiography is the mainstay in the diagnostic evaluation of constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM), but no single echocardiographic parameter is sufficiently robust to accurately distinguish between the two conditions. The present review summarizes the recent advances in echocardiography that promise to improve its diagnostic performance for this purpose. The role of other imaging modalities such as cardiac computed tomography, magnetic resonance imaging, and invasive hemodynamic assessment in the overall diagnostic approach is also discussed briefly...
May 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28382084/diverse-phenotypic-expression-of-cardiomyopathies-in-a-family-with-tnni3-p-arg145trp-mutation
#6
Ji-Won Hwang, Mi-Ae Jang, Shin Yi Jang, Soo Hyun Seo, Moon-Woo Seong, Sung Sup Park, Chang-Seok Ki, Duk-Kyung Kim
Genetic diagnosis of cardiomyopathies is challenging, due to the marked genetic and allelic heterogeneity and the lack of knowledge of the mutations that lead to clinical phenotypes. Here, we present the case of a large family, in which a single TNNI3 mutation caused variable phenotypic expression, ranging from restrictive cardiomyopathy (RCMP) to hypertrophic cardiomyopathy (HCMP) to near-normal phenotype. The proband was a 57-year-old female with HCMP. Examining the family history revealed that her elder sister had expired due to severe RCMP...
March 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28374271/effect-of-preoperative-left-ventricular-diastolic-dysfunction-on-mid-term-outcomes-after-surgical-ventricular-restoration-for-ischemic-cardiomyopathy
#7
Koji Furukawa, Mitsuhiro Yano, Eisaku Nakamura, Masakazu Matsuyama, Masanori Nishimura, Katsuya Kawagoe, Kunihide Nakamura
OBJECTIVES: The impact of surgical ventricular restoration (SVR) on survival and major adverse cardiac events (MACEs) is still controversial. The purposes of this study were to analyze our surgical experience with SVR for ischemic cardiomyopathy and to determine the effect of preoperative left ventricular diastolic dysfunction on mid-term outcomes after SVR. METHODS AND RESULTS: Between April 2010 and May 2016, 19 patients underwent SVR. The mean age was 60 ± 11 years and the mean New York Heart Association functional class was 2...
April 4, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28371863/hypercontractile-mutant-of-ventricular-myosin-essential-light-chain-leads-to-disruption-of-sarcomeric-structure-and-function-and-results-in-restrictive-cardiomyopathy-in-mice
#8
Chen-Ching Yuan, Katarzyna Kazmierczak, Jingsheng Liang, Rosemeire Kanashiro-Takeuchi, Thomas C Irving, Aldrin V Gomes, Yihua Wang, Thomas P Burghardt, Danuta Szczesna-Cordary
Aims: The E143K (Glu→Lys) mutation in the myosin essential light chain (ELC) has been associated with restrictive cardiomyopathy (RCM) in humans, but the mechanisms that underlie the development of defective cardiac function are unknown. Using transgenic E143K-RCM mice, we sought to determine the molecular and cellular triggers of E143K-induced heart remodeling. Methods and Results: The E143K-induced abnormalities in cardiac function and morphology observed by echocardiography and invasive hemodynamics were paralleled by augmented active and passive tension measured in skinned papillary muscle fibers compared with wild-type (WT)-generated force...
March 23, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28369760/clinical-genetic-testing-in-pediatric-cardiomyopathy-is-bigger-better
#9
Allison Chang Ouellette, Jacob Mathew, Ashok Kumar Manickaraj, George Manase, Laura Zahavich, Judith Wilson, Kristen George, Lee Benson, Sarah Bowdin, Seema Mital
For clinical genetic testing of cardiomyopathy (CMP), current guidelines do not address which gene panels to use: targeted panels specific to a CMP phenotype or expanded (panCMP) panels that include genes associated with multiple phenotypic subtypes. Our objective was to assess the clinical utility of targeted versus panCMP panel testing in pediatric CMPs. 151 pediatric patients with primary hypertrophic (n = 66), dilated (n = 64), restrictive (n = 8), or left-ventricular non-compaction (n = 13) CMP who underwent clinical genetic panel testing at a single centre were included...
March 30, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28364177/magnetic-resonance-imaging-for-characterizing-myocardial-diseases
#10
REVIEW
Maythem Saeed, Hui Liu, Chang-Hong Liang, Mark W Wilson
The National Institute of Health defined cardiomyopathy as diseases of the heart muscle. These myocardial diseases have different etiology, structure and treatment. This review highlights the key imaging features of different myocardial diseases. It provides information on myocardial structure/orientation, perfusion, function and viability in diseases related to cardiomyopathy. The standard cardiac magnetic resonance imaging (MRI) sequences can reveal insight on left ventricular (LV) mass, volumes and regional contractile function in all types of cardiomyopathy diseases...
March 31, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28356264/screening-of-the-filamin-c-gene-in-a-large-cohort-of-hypertrophic-cardiomyopathy-patients
#11
Juan Gómez, Rebeca Lorca, Julian R Reguero, César Morís, María Martín, Salvador Tranche, Belén Alonso, Sara Iglesias, Victoria Alvarez, Beatriz Díaz-Molina, Pablo Avanzas, Eliecer Coto
BACKGROUND: Recent exome sequencing studies identified filamin C (FLNC) as a candidate gene for hypertrophic cardiomyopathy (HCM). Our aim was to determine the rate of FLNC candidate variants in a large cohort of HCM patients who were also sequenced for the main sarcomere genes. METHODS AND RESULTS: A total of 448 HCM patients were next generation-sequenced (semiconductor chip technology) for the MYH7, MYBPC3, TNNT2, TNNI3, ACTC1, TNNC1, MYL2, MYL3, TPM1, and FLNC genes...
April 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28339731/obesity-associated-cardiac-pathogenesis-in-broiler-breeder-hens-development-of-metabolic-cardiomyopathy2-3
#12
C Y Chen, Y F Huang, Y J Ko, Y J Liu, Y H Chen, R L Walzem, S E Chen
Feed intake is typically restricted (R) in broiler hens to avoid obesity and improve egg production and livability. To determine whether improved heart health contributes to improved livability, fully adult 45-week-old R hens were allowed to consume feed to appetite (ad libitum; AL) up to 10 wk (70 d). Mortality, contractile functions, and morphology at 70 d, and measurements of cardiac hypertrophic remodeling at 7 d and 21 d were made and compared between R and AL hens. Outcomes for cardiac electrophysiology and mortality, reported separately, found increased mortality in AL hens in association with cardiac pathological hypertrophy and contractile dysfunction...
February 22, 2017: Poultry Science
https://www.readbyqxmd.com/read/28314540/technological-advances-shed-light-on-left-ventricular-cardiac-disturbances-in-cystic-fibrosis
#13
REVIEW
Zahra N Sayyid, Zachary M Sellers
Cystic fibrosis (CF), the most common autosomal recessive lethal disease in Caucasians, causes chronic pulmonary disease and can lead to cor pulmonale with right ventricular dysfunction. The presence of the cystic fibrosis transmembrane conductance regulator (CFTR) in cardiac myocardia has prompted debate regarding possible defective ion channel-induced cardiomyopathy. Clinical heart disease in CF is considered rare and is restricted to case reports. It has been unclear if this is due to the lack of physiological importance of CFTR in the heart, the relatively short lifespan of those with CF, or a technical inability to detect subclinical disease...
March 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28290973/total-artificial-heart-implantation-as-a-bridge-to-heart-transplantation-in-an-active-duty-service-member-with-amyloid-cardiomyopathy
#14
Michael S Scully, Dylan E Wessman, James M McKee, Gregory M Francisco, Keshav R Nayak, Jon A Kobashigawa
INTRODUCTION: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28285268/cardiac-manifestations-of-parasitic-diseases
#15
REVIEW
Maria Carmo P Nunes, Milton Henriques Guimarães Júnior, Adriana Costa Diamantino, Claudio Leo Gelape, Teresa Cristina Abreu Ferrari
The heart may be affected directly or indirectly by a variety of protozoa and helminths. This involvement may manifest in different ways, but the syndromes resulting from impairment of the myocardium and pericardium are the most frequent. The myocardium may be invaded by parasites that trigger local inflammatory response with subsequent myocarditis or cardiomyopathy, as occurs in Chagas disease, African trypanosomiasis, toxoplasmosis, trichinellosis and infection with free-living amoebae. In amoebiasis and echinococcosis, the pericardium is the structure most frequently involved with consequent pericardial effusion, acute pericarditis, cardiac tamponade or constrictive pericarditis...
May 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28284183/multiparametric-cardiac-magnetic-resonance-imaging-cmr-for-the-diagnosis-of-loeffler-s-endocarditis-a-case-report
#16
Mareike Gastl, Patrick Behm, Christoph Jacoby, Malte Kelm, Florian Bönner
BACKGROUND: Endocarditis parietalis fibroplastica Löfflein (EPF) is a rare form of primary restrictive cardiomyopathy with poor prognosis. It is generally caused by hypereosinophilic syndrome with eosinophilic penetration of the heart. This leads to congestive heart failure in three different stages. As a frequent manifestation of neoplastic diseases, cardiac involvement means poor prognosis. CASE PRESENTATION: The present report deals with a case of EPF caused by non-specified T-cell lymphoma (T-NOS)...
March 11, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28247148/very-long-chain-acyl-coa-dehydrogenase-vlcad-deficiency-studies-on-treatment-effects-and-long-term-outcomes-in-mouse-models
#17
REVIEW
Sara Tucci
Very-long-chain-acyl-CoA-dehydrogenase deficiency is the most common disorder of mitochondrial long-chain fatty acid (LCFA) oxidation, with an incidence of 1:50,000-1:100,000 in newborns. Catabolic situations contribute to the aggravation of symptoms and induce severe metabolic derangement. Treatment for VLCAD-deficiency includes avoidance of fasting and a long-chain fat-restricted and fat-modified diet in which LCFAs are fully or partially replaced by medium-chain triglycerides (MCT). The aim of this work was to investigate the outcome and the effects of long-term treatment in a mouse model of VLCAD-deficiency...
February 28, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28244013/impact-of-airflow-limitation-in-chronic-heart-failure
#18
S Bektas, F M E Franssen, V van Empel, N Uszko-Lencer, J Boyne, C Knackstedt, H P Brunner-La Rocca
BACKGROUND: Comorbidities are common in chronic heart failure (HF) patients, but diagnoses are often not based on objective testing. Chronic obstructive pulmonary disease (COPD) is an important comorbidity and often neglected because of shared symptoms and risk factors. Precise prevalence and consequences are not well known. Therefore, we investigated prevalence, pulmonary treatment, symptoms and quality of life (QOL) of COPD in patients with chronic HF. METHODS: 205 patients with stable HF for at least 1 month, aged above 50 years, were included from our outpatient cardiology clinic, irrespective of left ventricular ejection fraction...
February 27, 2017: Netherlands Heart Journal
https://www.readbyqxmd.com/read/28225712/left-ventricular-function-and-exercise-performance-in-idiopathic-dilated-cardiomyopathy-role-of-tissue-doppler-imaging
#19
Elena Zambon, Annamaria Iorio, Concetta Di Nora, Cosimo Carriere, Elena Abate, Marco Merlo, Giulia Barbati, Andrea Di Lenarda, Bruno Pinamonti, Piergiuseppe Agostoni, Gianfranco Sinagra
BACKGROUND: To examine the relationship between left ventricular (LV) function evaluated at echocardiography and exercise performance in idiopathic dilated cardiomyopathy (IDCM) patients. METHODS AND RESULTS: We enrolled 76 consecutive IDCM patients in sinus rhythm, undergoing cardiopulmonary exercise testing and echocardiography [49 ± 13 years old; LV ejection fraction 31 ± 7%, LV end-diastolic volume 96 ± 31 ml/m; peak oxygen consumption (peak VO2/kg) 18 ± 5...
April 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/28203206/mild-and-short-term-caloric-restriction-prevents-obesity-induced-cardiomyopathy-in-young-zucker-rats-without-changing-in-metabolites-and-fatty-acids-cardiac-profile
#20
Gema Ruiz-Hurtado, Concha F García-Prieto, Helena Pulido-Olmo, Juan P Velasco-Martín, Palmira Villa-Valverde, María E Fernández-Valle, Lisardo Boscá, María Fernández-Velasco, Javier Regadera, Beatriz Somoza, María S Fernández-Alfonso
Caloric restriction (CR) ameliorates cardiac dysfunction associated with obesity. However, most of the studies have been performed under severe CR (30-65% caloric intake decrease) for several months or even years in aged animals. Here, we investigated whether mild (20% food intake reduction) and short-term (2-weeks) CR prevented the obese cardiomyopathy phenotype and improved the metabolic profile of young (14 weeks of age) genetically obese Zucker fa/fa rats. Heart weight (HW) and HW/tibia length ratio was significantly lower in fa/fa rats after 2 weeks of CR than in counterparts fed ad libitum...
2017: Frontiers in Physiology
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