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https://www.readbyqxmd.com/read/28933176/-myocarditis-and-inflammatory-cardiomyopathy
#1
Dalibor Mlejnek, Jan Krejčí
Myocarditis is an inflammation of the heart muscle. The most common cause of myocarditis is viral infection in industrialized countries. Myocarditis with left ventricular dysfunction is called inflammatory cardiomyopathy and is the major cause of dilated cardiomyopathy. The clinical picture is very diverse, most often the patient present with signs of heart failure, arrhythmic symptoms and chest pains. Despite significant advances in non-invasive diagnostics, particularly magnetic resonance imaging, endomyocardial biopsy remains the gold standard of myocarditis diagnosis...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#2
REVIEW
Teresa M Lee, Daphne T Hsu, Paul Kantor, Jeffrey A Towbin, Stephanie M Ware, Steven D Colan, Wendy K Chung, John L Jefferies, Joseph W Rossano, Chesney D Castleberry, Linda J Addonizio, Ashwin K Lal, Jacqueline M Lamour, Erin M Miller, Philip T Thrush, Jason D Czachor, Hiedy Razoky, Ashley Hill, Steven E Lipshultz
Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28912185/restrictive-cardiomyopathy-genetics-pathogenesis-clinical-manifestations-diagnosis-and-therapy
#3
REVIEW
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
https://www.readbyqxmd.com/read/28912179/classification-epidemiology-and-global-burden-of-cardiomyopathies
#4
REVIEW
William J McKenna, Barry J Maron, Gaetano Thiene
In the past 25 years, major advances were achieved in the nosography of cardiomyopathies, influencing the definition and taxonomy of this important chapter of cardiovascular disease. Nearly, 50% of patients dying suddenly in childhood or adolescence or undergoing cardiac transplantation are affected by cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic, restrictive, and noncompacted) and added to update the World Health Organization classification. Myocarditis has also been named inflammatory cardiomyopathy...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28906536/endomyocardial-fibrosis-in-sudan-clinical-and-echocardiographic-features
#5
Siddiq Ibrahim Khalil, Suha Khalil, Salma El Tigani, Hanan A Saad
OBJECTIVE: Endomyocardial fibrosis (EMF) is a rare disease and is often an underdiagnosed and forgotten cardiomyopathy. The objective of this study was to document the current frequency of EMF in Sudan by defining and selecting cases from patients attending the echocardiography laboratory. Additionally we aimed to create an EMF registry for Sudan. METHODS: The study started in January 2007 and is on-going. All the patients attending our echocardiography clinics in four different hospitals in Khartoum, Sudan, were included...
July 2017: Cardiovascular Journal of Africa
https://www.readbyqxmd.com/read/28906150/safety-and-efficacy-of-a-ttr-specific-antisense-oligonucleotide-in-patients-with-transthyretin-amyloid-cardiomyopathy
#6
Merrill D Benson, Noel R Dasgupta, Stacy M Rissing, Jessica Smith, Harvey Feigenbaum
OBJECTIVES: Cardiomyopathy is a major cause of death in both the hereditary form of transthyretin (TTR) amyloidosis and the sporadic late-age-onset transthyretin amyloidosis (ATTR wild-type (ATTRwt)). Clinically disease progression from time of diagnosis to death is usually quoted as 5- to 15-years. In prior studies, significant progression of cardiac parameters in patients with moderate to severe cardiomyopathy has been noted within a 12-month time span. METHODS: The present study was designed to prospectively monitor changes in cardiac parameters, both structural and functional, in patients with ATTR cardiomyopathy while treated with a TTR specific antisense oligonucleotide (ASO; IONIS-TTR℞) designed to lower blood levels of the amyloid fibril precursor protein...
September 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28906148/hip-and-knee-arthroplasty-are-common-among-patients-with-transthyretin-cardiac-amyloidosis-occurring-years-before-cardiac-amyloid-diagnosis-can-we-identify-affected-patients-earlier
#7
Jonah Rubin, Julissa Alvarez, Sergio Teruya, Adam Castano, Ronald A Lehman, Mark Weidenbaum, Jeffrey A Geller, Stephen Helmke, Mathew S Maurer
Transthyretin cardiac amyloidosis (ATTR-CA) causes a restrictive cardiomyopathy in older adults, often diagnosed at advanced stages when emerging therapies in late phase clinical trials may not have clinical benefit. This investigation aimed to detect clinical entities that may provide more advanced warning of ATTR-CA. Since ATTR preferentially deposits in ligaments, tendons, and articular cartilage, we hypothesized that ATTR-CA patients have a greater prevalence of total hip (THA) and knee (TKA) arthroplasties compared with the general population, and that arthroplasty occurs significantly before ATTR-CA diagnosis...
September 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28905470/outcomes-of-berlin-heart-excor-%C3%A2-pediatric-ventricular-assist-device-support-in-patients-with-restrictive-and-hypertrophic-cardiomyopathy
#8
Jennifer A Su, Jondavid Menteer
The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR(®) ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was reviewed. Patients with primary diastolic dysfunction are included in this study. Twenty pediatric patients with restrictive cardiomyopathy (n = 13), hypertrophic cardiomyopathy (n = 3), or congenital heart disease with restrictive physiology (n = 4) who were supported with EXCOR(®) were identified...
September 14, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28900547/chagas-cardiomyopathy-presenting-as-symptomatic-bradycardia-an-underappreciated-emerging-public-health-problem-in-the-united-states
#9
Richard Jesse Durrance, Tofura Ullah, Zulekha Atif, William Frumkin, Kaushik Doshi
Chagas cardiomyopathy (CCM) is traditionally considered a disease restricted to areas of endemicity. However, an estimated 300,000 people living in the United States today have CCM, of which its majority is undiagnosed. We present a case of CCM acquired in an endemic area and detected in its early stage. A 42-year-old El Salvadoran woman presented with recurrent chest pain and syncopal episodes. Significant family history includes a sister in El Salvador who also began suffering similar episodes. Physical exam and ancillary studies were only remarkable for sinus bradycardia...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28891481/cardiac-manifestations-in-children-with-inborn-errors-of-metabolism
#10
Kyriaki Papadopoulou-Legbelou, Maria Gogou, Athanasios Evangeliou
NEED AND PURPOSE: Cardiac involvement is a part of many inborn errors of metabolism, but has not been systematically studied. This review focuses on studies describing cardiac manifestations of inborn errors of metabolism in childhood. METHODS: Two independent reviewers searched the topic using PubMed database. Studies published within 20 years were considered, without applying any restrictions related to study design. Despite the small number of existing systematic studies on the topic, several case series/reports were identified...
August 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28890659/loeffler-endocarditis-a-unique-presentation-of-right-sided-heart-failure-due-to-eosinophil-induced-endomyocardial-fibrosis
#11
Amit Alam, Shankar Thampi, Shahryar G Saba, Rita Jermyn
Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention. We present a unique case of a woman with remote history of hypereosinophilic syndrome, attributed to treatment of rheumatoid arthritis with infliximab, who presented with symptoms of heart failure refractory to medical management and was found to have Loeffler endocarditis...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/28887775/imaging-cardiac-innervation-in-amyloidosis
#12
Riemer H J A Slart, Andor W J M Glaudemans, Bouke P C Hazenberg, Walter Noordzij
Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. The use of iodine-123 labelled metaiodobenzylguanidine ([I-123]MIBG), a chemical modified analogue of norepinephrine, is well established in patients with heart failure and plays an important role in evaluation of sympathetic innervation in cardiac amyloidosis...
September 8, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28885342/clinical-features-of-idiopathic-restrictive-cardiomyopathy-a-retrospective-multicenter-cohort-study-over-2-decades
#13
MULTICENTER STUDY
Jung Ae Hong, Min-Seok Kim, Min-Su Cho, Hyo In Choi, Duk-Hyun Kang, Sang-Eun Lee, Ga-Yeon Lee, Eun-Seok Jeon, Jae-Yeong Cho, Kye-Hun Kim, Byung-Su Yoo, Jong-Young Lee, Won-Jang Kim, Kyung-Hee Kim, Wook-Jin Chung, Ju-Hee Lee, Myeong-Chan Cho, Jae-Joong Kim
Idiopathic restrictive cardiomyopathy (RCMP) has not been fully understood because this disease is difficult to diagnose. The present study aimed to assess the clinical profile and outcome of idiopathic RCMP from a multicenter cohort.This investigation is a retrospective study of consecutive patients with idiopathic RCMP at 10 centers in Korea between 1990 and 2010. We evaluated the clinical characteristics of the patients and prognostic factors associated with mortality using multivariate Cox proportional hazards regression analyses...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28861149/posttranslational-modifications-of-calcium-calmodulin-dependent-protein-kinase-ii%C3%AE-and-its-downstream-signaling-in-human-failing-hearts
#14
Tomas Rajtik, Eva Goncalvesova, Zoltan V Varga, Przemyslaw Leszek, Mariusz Kusmierczyk, Michal Hulman, Jan Kyselovic, Peter Ferdinandy, Adriana Adameova
BACKGROUND: In human failing hearts (HF) of different origin (coronary artery disease-CAD, dilated-DCM, restrictive and hypertrophic cardiomyopathy-OTHER), we investigated the active forms of Ca(2+)/calmodulin-dependent protein kinase IIδ (p-Thr(287)-CaMKIIδ, oxMet(281/282)-CaMKIIδ) and their role in phenotypes of the disease. METHODS AND RESULTS: Although basic diagnostic and clinical markers indicating the attenuated cardiac contractility and remodeling were comparable in HF groups, CaMKIIδ-mediated axis was different...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28831623/structural-consequences-of-mutations-associated-with-idiopathic-restrictive-cardiomyopathy
#15
Svetlana Tarnovskaya, Artem Kiselev, Anna Kostareva, Dmitrij Frishman
Idiopathic restrictive cardiomyopathy (RCM, MIM# 115210) is the least common type of cardiomyopathies, often of genetic origin. Recently we described a spectrum of variants-classified as pathogenic, likely pathogenic and variants of unknown significance-in 24 patients suffering from idiopathic RCM. Pathogenic variants, detected in half of the RCM cases, were found in sarcomeric and cytoskeletal genes that have a predominant role in the development of RCM. Here we have analyzed the structural consequences of these missense variants and predicted their effect on the function of three large groups of domains: intrinsically disordered regions (IDRs), fibronectin-type III (FnIII) domains, and immunoglobulin-like (Ig) domains...
August 22, 2017: Amino Acids
https://www.readbyqxmd.com/read/28821499/primary-ventricular-tachycardia-in-paediatric-population-in-a-tertiary-centre
#16
Shuenn-Nan Chiu, Wei-Lun Wu, Chun-Wei Lu, Wei-Chieh Tseng, Kun-Lang Wu, Jou-Kou Wang, Mei-Hwan Wu
OBJECTIVE: To delineate the outcome of ventricular tachycardia (VT) in the paediatric population. METHODS: Patients who developed sustained VT between the ages of 0 and 18 years in a referral centre from 1991 to 2015 were enrolled. RESULTS: A total of 116 patients (67 male/49 female) had documented VT, and 53 (46%) had associated heart disease, namely cardiomyopathy in 20 (17%), structural heart disease in 19 (16%) and channelopathy in 14 (12%), and some of them presented with two types of associated heart disease...
August 18, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28820624/retinopathy-and-optic-atrophy-expanding-the-phenotypic-spectrum-of-pathogenic-variants-in-the-aars2-gene
#17
Jason H Peragallo, Stephanie Keller, Marjo S van der Knaap, Bruno P Soares, Suma P Shankar
BACKGROUND: Optic atrophy may be the sequela of optic nerve injury due to any insult, including isolated and syndromic genetic diseases. Alanyl-tRNA synthetase 2 (AARS2) pathogenic variants have been reported to cause leukodystrophy with ovarian failure, and cardiomyopathy (#615889) as well as combined oxidative phosphorylation deficiency-8 (#614096). We report a young child who presented with decreased vision due to optic atrophy and was found to harbor missense variants in the AARS2 gene expanding the phenotypic expression of the AARS2 gene...
August 18, 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28797094/whole-gene-sequencing-identifies-deep-intronic-variants-with-potential-functional-impact-in-patients-with-hypertrophic-cardiomyopathy
#18
Rita Mendes de Almeida, Joana Tavares, Sandra Martins, Teresa Carvalho, Francisco J Enguita, Dulce Brito, Maria Carmo-Fonseca, Luís Rocha Lopes
BACKGROUND: High throughput sequencing technologies have revolutionized the identification of mutations responsible for genetic diseases such as hypertrophic cardiomyopathy (HCM). However, approximately 50% of individuals with a clinical diagnosis of HCM have no causal mutation identified. This may be due to the presence of pathogenic mutations located deep within the introns, which are not detected by conventional sequencing analysis restricted to exons and exon-intron boundaries. OBJECTIVE: The aim of this study was to develop a whole-gene sequencing strategy to prioritize deep intronic variants that may play a role in HCM pathogenesis...
2017: PloS One
https://www.readbyqxmd.com/read/28793004/abnormal-diastolic-function-underlies-the-different-beneficial-effects-of-cardiac-resynchronization-therapy-on-ischemic-and-non-ischemic-cardiomyopathy
#19
Qi Wang, Kang-Yu Chen, Fei Yu, Hao Su, Chun-Sheng An, Yang Hu, Dong-Mei Yang, Jian Xu, Ji Yan
OBJECTIVES: To investigate the association between diastolic function and the different beneficial effects of cardiac resynchronization therapy in patients with heart failure due to different causes. METHODS: The 104 enrolled patients were divided into an ischemic cardiomyopathy group (n=27) and a non-ischemic cardiomyopathy group (n=77) according to the cause of heart failure. Before implantation, left ventricular diastolic function was evaluated in all patients using echocardiography...
July 2017: Clinics
https://www.readbyqxmd.com/read/28772037/cardiac-and-peripheral-autonomic-control-in-restrictive-cardiomyopathy
#20
Ana Luiza C Sayegh, Marcelo R Dos Santos, Adriana O Sarmento, Francis R de Souza, Vera M C Salemi, Viviane T Hotta, Akothirene Cristhina D B Marques, Heidrum H Krämer, Ivani C Trombetta, Charles Mady, Maria Janieire de Nazaré Nunes Alves
AIMS: Autonomic dysfunction determines the advance of dilated cardiomyopathy (DCM) and is related to poor outcomes. However, this autonomic imbalance is unknown in patients with restrictive cardiomyopathy (RCM) even though they have similar symptoms and poor quality of life as DCM patients have. The aim of this study was to evaluate if autonomic and neurovascular controls were altered in RCM patients. METHODS AND RESULTS: Fifteen RCM patients, 10 DCM patients, and 10 healthy subjects were evaluated...
August 2017: ESC Heart Failure
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