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https://www.readbyqxmd.com/read/29747658/naxos-disease-from-the-origin-to-today
#1
REVIEW
Guo-Liang Li, Ardan M Saguner, Guy H Fontaine
Naxos disease, first described by Dr. Nikos Protonotarios and colleagues on the island of Naxos, Greece, is a special form of arrhythmogenic right ventricular dysplasia (ARVD). It is an inherited condition with a recessive form of transmission and a familial penetrance of 90%. It is associated with thickening of the skin of the hands and sole, and a propensity to woolly hair. The cardiac anomalies characterized by ventricular arrhythmias with ventricular extrasystoles and tachycardia and histologic features of the myocardium are consistent with ARVD, but in a more severe form of dysplasia with major dilatation of the right ventricle...
May 10, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29688316/diagnostic-and-therapeutic-strategies-for-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-patient
#2
Weijia Wang, Cynthia A James, Hugh Calkins
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare inherited heart muscle disease characterized by ventricular tachyarrhythmia, predominant right ventricular dysfunction, and sudden cardiac death. Its pathophysiology involves close interaction between genetic mutations and exposure to physical activity. Mutations in genes encoding desmosomal protein are the most common genetic basis. Genetic testing plays important roles in diagnosis and screening of family members. Syncope, palpitation, and lightheadedness are the most common symptoms...
April 23, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29603270/value-of-a-novel-16-lead-high-definition-ecg-machine-to-detect-conduction-abnormalities-in-structural-heart-disease
#3
Guo-Liang Li, Ardan M Saguner, Deniz Akdis, Guy Hugues Fontaine
BACKGROUND: Depolarization abnormalities are hardly detectable by standard 12-lead ECG in some patients. OBJECTIVE: To evaluate the value of the 16-lead HD-ECG machine to record conduction abnormalities including Epsilon waves in patients with structural heart disease. METHODS: Tracings with 12-lead ECG, 16-lead HD-ECG and SAECG were studied. RESULTS: 1. Case of severe CAD On 16-lead HD-ECG, a tiny intra QRS signal was noted in lead-III, a prolonged P wave in lead-II, and fragmentation on top of lead-aVL and lead-aVF...
March 30, 2018: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29574980/blockade-of-the-renin-angiotensin-aldosterone-system-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-a-double-blind-multicenter-prospective-randomized-genotype-driven-study-brave-study
#4
Elodie Morel, Ab Waheed Manati, Patrice Nony, Delphine Maucort-Boulch, Francis Bessière, Xu Cai, Timothee Besseyre des Horts, Alexandre Janin, Adrien Moreau, Phillippe Chevalier
Arrhythmogenic right ventricular dysplasia (ARVD) is a rare cardiomyopathy characterized by the progressive replacement of cardiomyocytes by fatty and fibrous tissue in the right ventricle (RV). These infiltrations lead to cardiac electrical instability and ventricular arrhythmia. Current treatment for ARVD is empirical and essentially based on treatment of arrhythmia. Thus, there is no validated treatment that will prevent the deterioration of RV function in patients with ARVD. The aim of the BRAVE study is to evaluate the effect of ramipril, an angiotensin-converting enzyme inhibitor, on ventricular myocardial remodeling and arrhythmia burden in patients with ARVD...
March 25, 2018: Clinical Cardiology
https://www.readbyqxmd.com/read/29524887/lc-ms-ms-determination-of-antiretroviral-drugs-in-influents-and-effluents-from-wastewater-treatment-plants-in-kwazulu-natal-south-africa
#5
Ovokeroye A Abafe, Jana Späth, Jerker Fick, Stina Jansson, Chris Buckley, Annegret Stark, Bjoern Pietruschka, Bice S Martincigh
South Africa has the largest occurrence of the human immune deficiency virus (HIV) in the world but has also implemented the largest antiretroviral (ARV) treatment programme. It was therefore of interest to determine the presence and concentrations of commonly used antiretroviral drugs (ARVDs) and, also, to determine the capabilities of wastewater treatment plants (WWTPs) for removing ARVDs. To this end, a surrogate standard based LC-MS/MS method was optimized and applied for the detection of thirteen ARVDs used in the treatment and management of HIV/acquired immune deficiency syndrome (HIV/AIDS) in two major and one modular WWTP in the eThekwini Municipality in KwaZulu-Natal, South Africa...
June 2018: Chemosphere
https://www.readbyqxmd.com/read/29383263/intracardiac-ultrasound-to-detect-aneurysm-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#6
Cismaru Gabriel, Mihai Puiu, Radu Rosu, Lucian Muresan, Raluca Rancea, Gabriel Gusetu, Dana Pop, Dumitru Zdrenghea
Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a genetic disorder characterized by fibrofattty replacement of the right ventricular myocardium. In the revised 2010 Task Force Criteria, a major criteria for ARVD/C is the presence of RV aneurysm by 2D echo. Our report demonstrates that intracardiac ultrasound can detect RV aneurysms and also focal absence of trabeculations which brings additional value to the diagnosis of ARVD/C. A 26-year-old male patient suffering from multiple sustained episodes of ventricular tachycardia was implanted with an internal cardiac defibrillator after confirmation of the disease by cardiac magnetic resonance imaging...
January 2018: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29178656/unique-genetic-background-and-outcome-of-non-caucasian-japanese-probands-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#7
Yuko Wada, Seiko Ohno, Takeshi Aiba, Minoru Horie
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy mainly caused by desmosomal gene mutation. More than half of Caucasian probands have desmosomal mutations, which lead to earlier onset of ventricular arrhythmias. Among non-Caucasians, the genetic background of ARVD/C probands and its prognostic impact remain unclear. METHODS AND RESULTS: We genotyped 99 unrelated Japanese ARVD/C probands for plakophilin 2 (PKP2), desmoglein 2 (DSG2), desmoplakin (DSP), and desmocollin 2 (DSC2) between 2005 and 2014...
November 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29060659/multi-view-collaborative-segmentation-for-prostate-mri-images
#8
Xiuying Wang, Wensi Tang, Hui Cui, Shan Zeng, David Dagan Feng, Michael Fulham
Prostate delineation from MRI images is a prolonged challenging issue partially due to appearance variations across patients and disease progression. To address these challenges, our proposed collaborative method takes into account the computed multiple label-relevance maps as multiple views for learning the optimal boundary delineation. In our method, we firstly extracted multiple label-relevance maps to represent the affinities between each unlabeled pixel to the pre-defined labels to avoid the selection of handcrafted features...
July 2017: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/29056991/atherosclerotic-renovascular-disease-epidemiology-treatment-and-current-challenges
#9
REVIEW
Diana Vassallo, Philip A Kalra
The neutral results of recent large randomized controlled trials comparing renal revascularization with optimal medical therapy in patients with atherosclerotic renovascular disease (ARVD) have cast doubt on the role of revascularization in the management of unselected patients with this condition. However, these studies have strengthened the evidence base for the role of contemporary intensive medical vascular protection therapy and aggressive risk factor control in improving clinical outcomes in ARVD. Patients presenting with 'high-risk' clinical features such as uncontrolled hypertension, rapidly declining renal function or flash pulmonary oedema are underrepresented in these studies; hence these results may not be applicable to all patients with ARVD...
2017: Postępy W Kardiologii Interwencyjnej, Advances in Interventional Cardiology
https://www.readbyqxmd.com/read/29029613/right-precordial-directed-electrocardiographical-markers-identify-arrhythmogenic-right-ventricular-cardiomyopathy-in-the-absence-of-conventional-depolarization-or-repolarization-abnormalities
#10
Daniel Cortez, Anneli Svensson, Jonas Carlson, Sharon Graw, Nandita Sharma, Francesca Brun, Anita Spezzacatene, Luisa Mestroni, Pyotr G Platonov
BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries a risk of sudden death. We aimed to assess whether vectorcardiographic (VCG) parameters directed toward the right heart and a measured angle of the S-wave would help differentiate ARVD/C with otherwise normal electrocardiograms from controls. METHODS: Task Force 2010 definite ARVD/C criteria were met for all patients. Those who did not fulfill Task Force depolarization or repolarization criteria (-ECG) were compared with age and gender-matched control subjects...
October 13, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/29024127/pacemapping-guided-by-defibrillator-electrograms-for-ventricular-tachycardia-ablation-in-a-patient-with-arvd-c
#11
Adolfo Fontenla, Álvaro Lozano, María López-Gil, Fernando Arribas
No abstract text is available yet for this article.
February 2018: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29016773/multichannel-electrocardiogram-diagnostics-for-the-diagnosis-of-arrhythmogenic-right-ventricular-dysplasia
#12
Ricards Marcinkevics, James O'Neill, Hannah Law, Eleftheria Pervolaraki, Andrew Hogarth, Craig Russell, Berthold Stegemann, Arun V Holden, Muzahir H Tayebjee
Aims: The identification of arrhythmogenic right ventricular dysplasia (ARVD) from 12-channel standard electrocardiogram (ECG) is challenging. High density ECG data may identify lead locations and criteria with a higher sensitivity. Methods and results: Eighty-channel ECG recording from patients diagnosed with ARVD and controls were quantified by magnitude and integral measures of QRS and T waves and by a measure (the average silhouette width) of differences in the shapes of the normalized ECG cycles...
August 29, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28979908/epicardial-ablation-of-ventricular-tachycardia-in-a-patient-with-arrhythmogenic-right-ventricular-dysplasia-after-failed-conventional-endocardial-ablation-a-case-for-remote-navigation-with-functional-image-integration
#13
Sabine Ernst, Karine Roy, Eric Lim, Glyn Thomas
Arrhythmogenic right ventricular dysplasia (ARVD) is an inheritable heart muscle disease that predominantly affects the right ventricle (RV) and predisposes to ventricular arrhythmias and sudden cardiac death (SCD)(1). The natural history is predominantly related to ventricular electric instability which may lead to arrhythmic SCD, mostly in young people and athletes(2,3), but may progress with significant RV muscle disease and left-ventricular (LV) involvement and can result in right or biventricular heart failure(4)...
December 30, 2016: Global Cardiology Science & Practice
https://www.readbyqxmd.com/read/28863780/feature-tracking-cmr-reveals-abnormal-strain-in-preclinical-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-a-multisoftware-feasibility-and-clinical-implementation-study
#14
Mimount Bourfiss, Davis M Vigneault, Mounes Aliyari Ghasebeh, Brittney Murray, Cynthia A James, Crystal Tichnell, Firdaus A Mohamed Hoesein, Stefan L Zimmerman, Ihab R Kamel, Hugh Calkins, Harikrishna Tandri, Birgitta K Velthuis, David A Bluemke, Anneline S J M Te Riele
BACKGROUND: Regional right ventricular (RV) dysfunction is the hallmark of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), but is currently only qualitatively evaluated in the clinical setting. Feature Tracking Cardiovascular Magnetic Resonance (FT-CMR) is a novel quantitative method that uses cine CMR to calculate strain values. However, most prior FT-CMR studies in ARVD/C have focused on global RV strain using different software methods, complicating implementation of FT-CMR in clinical practice...
September 1, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28705047/galectin-3-correlates-with-arrhythmogenic-right-ventricular-cardiomyopathy-and-predicts-the-risk-of-ventricular-arrhythmias-in-patients-with-implantable-defibrillators
#15
Fahrettin Oz, Imran Onur, Ali Elitok, Evin Ademoglu, Ibrahim Altun, Ahmet Kaya Bilge, Kamil Adalet
Background Arrhythmogenic right ventricular dysplasia (ARVD) is a heritable disorder characterized by fibro-fatty replacement of right ventricular myocytes, increased risk of ventricular arrhythmias, and sudden cardiac death. Galectin-3 (GAL3) is known to play an important role in a number of fibrotic conditions, including cardiac fibrosis. Many studies have focused on the association between GAL3 levels and cardiac fibrosis in heart failure. However, the role of GAL3 in the pathogenesis of ARVD and ventricular arrhythmias has not yet been evaluated thoroughly...
August 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28595345/supraventricular-arrhythmias-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-associate-with-long-term-outcome-after-catheter-ablation-of-ventricular-tachycardias
#16
Andreas Müssigbrodt, Helge Knopp, Elena Efimova, Alexander Weber, Livio Bertagnolli, Sebastian Hilbert, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
Aims: This study aimed to assess the impact of supraventricular tachycardia (SVT) on long-term results of radiofrequency catheter ablation therapy of ventricular tachycardia (VT) in a large cohort of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Methods and results: Supraventricular tachycardia occurrence has been studied in patients from our ARVD/C registry (70 patients, 48 male, age 53.2 ± 14.0, 45 patients (64.3%) with previous VT ablation)...
June 7, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28594847/kidney-volume-to-gfr-ratio-predicts-functional-improvement-after-revascularization-in-atheromatous-renal-artery-stenosis
#17
Constantina Chrysochou, Darren Green, James Ritchie, David L Buckley, Philip A Kalra
BACKGROUND: Randomized controlled trials (RCT) have shown no overall benefit of renal revascularization in atherosclerotic renovascular disease (ARVD). However, 25% of patients demonstrate improvement in renal function. We used the ratio of magnetic resonance parenchymal volume (PV) to isotopic single kidney glomerular filtration rate (isoSKGFR) ratio as our method to prospectively identify "improvers" before revascularization. METHODS: Patients with renal artery stenosis who were due revascularization were recruited alongside non-ARVD hypertensive CKD controls...
2017: PloS One
https://www.readbyqxmd.com/read/28536891/myocardial-voltage-ratio-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#18
REVIEW
Andreas Müssigbrodt, Livio Bertagnolli, Elena Efimova, Jedrzej Kosiuk, Borislav Dinov, Kerstin Bode, Simon Kircher, Nikolaos Dagres, Michael Döring, Sergio Richter, Philipp Sommer, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
AIMS: This study aimed to analyze the influence of scar distribution between the endocardium and the epicardium in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). METHODS: Electroanatomical mapping data were derived from our ARVD/C registry. Myocardial voltage distribution between the endocardium and the epicardium was analyzed in 28 patients (18 men, 49.9 ± 13.0 years) with previous ventricular tachycardia (VT) ablation and complete right ventricular maps...
June 2017: Herzschrittmachertherapie & Elektrophysiologie
https://www.readbyqxmd.com/read/28523642/autosomal-recessive-nonsyndromic-arrhythmogenic-right-ventricular-cardiomyopathy-without-cutaneous-involvements-a-novel-mutation
#19
REVIEW
Mahdieh Soveizi, Bahareh Rabbani, Yousef Rezaei, Sedigheh Saedi, Nasim Najafi, Majid Maleki, Nejat Mahdieh
The arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic disease frequently associated with desmosomal mutations, mainly attributed to dominant mutations in the Plakophilin-2 (PKP2) gene. Naxos and Carvajal are the syndromic forms of ARVD/C due to recessive mutations. Herein, we report an autosomal recessive form of nonsyndromic ARVD/C caused by a mutation in the PKP2 gene. After examination and implementation of diagnostic modalities, the definite diagnosis of ARVD/C was confirmed by detection of ventricular tachycardia with a left bundle branch configuration and a superior axis, T-wave inversion in right precordial leads (i...
July 2017: Annals of Human Genetics
https://www.readbyqxmd.com/read/28509892/arrhythmogenic-right-ventricular-dysplasia-an-under-recognized-form-of-inherited-cardiomyopathy
#20
George O Adesina, Shelly A Hall, Jose C Mendez, Susan M Joseph, Robert L Gottlieb, Parag P Kale, Amarinder S Bindra
We report a case of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD) in order to evaluate the course of an under-recognized form of cardiomyopathy with a vast array of clinical manifestations. The patient is a 49-year-old white woman transferred from an outside hospital due to dyspnea and persistent hypoxia. She had a pertinent family history that included a sister who died suddenly in her 30s from unexplained heart failure. Initial work-up for hypoxia was unrevealing. Transthoracic echocardiography revealed isolated right ventricular dysfunction with dilation and multiple trabeculations...
2017: Reviews in Cardiovascular Medicine
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