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https://www.readbyqxmd.com/read/29225258/isolated-cardiac-sarcoidosis-mimicking-arrhythmogenic-right-ventricular-cardiomyopathy
#1
Hirotaka Waki, Kazuo Eguchi, Shinichi Toriumi, Tomokazu Ikemoto, Tsukasa Suzuki, Noriyoshi Fukushima, Kazuomi Kario
The diagnosis of cardiac sarcoidosis (CS) has become easier due to advances in imaging modalities, but we sometimes encounter difficult-to-diagnose patients. We herein report the case of a 60-year-old Japanese woman who was diagnosed with isolated CS, although she also met the diagnostic criteria of arrhythmogenic right ventricular cardiomyopathy (ARVC). A histological examination by an endomyocardial biopsy of the right ventricle revealed the typical findings of granulomatous change for CS. Although she did not show any characteristics of systemic sarcoidosis, oral prednisolone treatment was introduced, and she achieved a good response...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29188317/genetic-testing-in-pediatric-cardiomyopathy
#2
Chalani D Ellepola, Linda M Knight, Peter Fischbach, Shriprasad R Deshpande
Genetic testing is recommended in patients with dilated cardiomyopathy (DCM); however, limited studies demonstrate high yields of genetic testing in non-hypertrophic (HCM) patients. Furthermore, there is sparse genotype-phenotype data in pediatric DCM patients. We performed a retrospective review of 70 consecutive probands with cardiomyopathy (non-HCM) who underwent genetic evaluation. Mean age at presentation was 5.48 years. Echocardiography revealed mean ejection fraction of 32.4%. The LVEDd z score ranged from - 5...
November 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29177016/-caterpillar-right-ventricle-unusual-manifestation-of-a-rare-disease
#3
Suneesh Kalliath, Gopalan Nair Rajesh
A 66-year-old man presented with abdominal distension and pedal oedema for the past 10 years. He had history of right heart failure on several occasions in the past, and one of these admissions prompted referral to a cardiac specialist. On examination, he had markedly elevated jugular venous pressure with prominent 'y-descent', a tricuspid regurgitation murmur, gross ascites and pedal oedema. A full blood count, routine biochemical screen and serum bicarbonate levels were normal. Right ventricular angiogram revealed a 'caterpillar'-like aneurysm of the right ventricle (RV) apex (figure 1 arrow) with a dilated right ventricular outflow tract and significant tricuspid regurgitation with a dilated right atrium (see online supplementary video 1)...
2017: Heart Asia
https://www.readbyqxmd.com/read/29173404/sudden-cardiac-death-in-genetic-cardiomyopathies
#4
REVIEW
Gourg Atteya, Rachel Lampert
Sudden cardiac death (SCD) caused by ventricular arrhythmias is common in patients with genetic cardiomyopathies (CMs) including dilated CM, hypertrophic CM, and arrhythmogenic right ventricular CM (ARVC). Phenotypic features can identify individuals at high enough risk to warrant placement of an implantable cardioverter-defibrillator, although risk stratification schemes remain imperfect. Genetic testing is valuable for family cascade screening but with few exceptions (eg, LMNA mutations) do not identify higher risk for SCD...
December 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/29158215/risk-stratification-in-arrhythmogenic-right-ventricular-cardiomyopathy
#5
REVIEW
Hugh Calkins, Domenico Corrado, Frank Marcus
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by ventricular arrhythmias and an increased risk of sudden cardiac death. Although structural abnormalities of the right ventricle predominate, it is well recognized that left ventricular involvement is common, particularly in advanced disease, and that left-dominant forms occur. The pathological characteristic of ARVC is myocyte loss with fibrofatty replacement. Since the first detailed clinical description of the disorder in 1982, significant advances have been made in understanding this disease...
November 21, 2017: Circulation
https://www.readbyqxmd.com/read/29155786/isolation-and-cultivation-of-adult-rat-cardiomyocytes
#6
Franziska Nippert, Rolf Schreckenberg, Klaus-Dieter Schlüter
In an intact heart, adjacent cells influence adult cardiomyocytes. With the method of isolation and cultivation of adult cardiomyocytes, a precise investigation of the behavior of these cells under specific treatments and environments is possible. This manuscript presents a protocol for successful isolation and cultivation of adult rat ventricular cardiomyocytes (ARVC). The rat is sacrificed by cervical dislocation under deep anesthesia. Then, the heart is extracted and the aorta is uncovered. Subsequently, perfusion on the Langendorff perfusion system with calcium depletion and collagenase treatment is performed...
October 19, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29107359/heart-transplantation-in-arrhythmogenic-right-ventricular-cardiomyopathy-experience-from-the-nordic-arvc-registry
#7
Thomas Gilljam, Kristina H Haugaa, Henrik K Jensen, Anneli Svensson, Henning Bundgaard, Jim Hansen, Göran Dellgren, Finn Gustafsson, Hans Eiskjær, Arne K Andreassen, Johan Sjögren, Thor Edvardsen, Anders G Holst, Jesper Hastrup Svendsen, Pyotr G Platonov
OBJECTIVE: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants. METHODS: Data from 31 ARVC/HTx patients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46years (14-65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry...
October 21, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29105955/right-ventricular-systolic-function-and-mechanical-dispersion-identify-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#8
Meriam Åström Aneq, Eva Maret, Lars Brudin, Anneli Svensson, Jan Engvall
PURPOSE: To assess right ventricular (RV) regional and global systolic function using feature tracking (FT) in patients with a definite diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) and to investigate if changes in strain amplitude and mechanical dispersion indicate a propensity for arrhythmia. MATERIALS AND METHODS: Twenty-seven patients fulfilling Task Force Criteria for ARVC and 24 healthy volunteers underwent MR at 1·5 Tesla. Steady-state free precession cine of long-axis slices and a short-axis stack of the RV was acquired...
November 6, 2017: Clinical Physiology and Functional Imaging
https://www.readbyqxmd.com/read/29090492/late-potentials-and-their-correlation-with-ventricular-structure-in-patients-with-ventricular-arrhythmias
#9
Peter Marstrand, Anna Axelsson, Jens Jakob Thune, Niels Vejlstrup, Steen Pehrson, Henning Bundgaard, Juliane Theilade
INTRODUCTION: The presence of late potentials (LP) may indicate a predisposition to ventricular arrhythmias and sudden cardiac death. We investigated the association between presence of LP and structural cardiac anomalies assessed by magnetic resonance (CMR) in patients presenting with ventricular arrhythmias. METHODS: We included 42 patients admitted with ventricular tachycardia or fibrillation who had undergone both signal-averaged ECG recording and CMR imaging...
November 1, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/29069369/high-resolution-three-dimensional-late-gadolinium-enhanced-cardiac-magnetic-resonance-imaging-to-identify-the-underlying-substrate-of-ventricular-arrhythmia
#10
Alexia Hennig, Marjorie Salel, Frederic Sacher, Claudia Camaioni, Soumaya Sridi, Arnaud Denis, Michel Montaudon, François Laurent, Pierre Jais, Hubert Cochet
Aims: Cardiac magnetic resonance (CMR) is recommended as a second-line method to diagnose ventricular arrhythmia (VA) substrate. We assessed the diagnostic yield of CMR including high-resolution late gadolinium-enhanced (LGE) imaging. Methods and results: Consecutive patients with sustained ventricular tachycardia (VT), non-sustained VT (NSVT), or ventricular fibrillation/aborted sudden death (VF/SCD) underwent a non-CMR diagnostic workup according to current guidelines, and CMR including LGE imaging with both a conventional breath-held and a free-breathing method enabling higher spatial resolution (HR-LGE)...
October 23, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/29062102/arrhythmogenic-cardiomyopathy-related-dsg2-mutations-affect-desmosomal-cadherin-binding-kinetics
#11
Mareike Dieding, Jana Davina Debus, Raimund Kerkhoff, Anna Gaertner-Rommel, Volker Walhorn, Hendrik Milting, Dario Anselmetti
Cadherins are calcium dependent adhesion proteins that establish the intercellular mechanical contact by bridging the gap to adjacent cells. Desmoglein-2 (Dsg2) is a specific cadherin of the cell-cell contact in cardiac desmosomes. Mutations in the DSG2-gene are regarded to cause arrhythmogenic (right ventricular) cardiomyopathy (ARVC) which is a rare but severe heart muscle disease. The molecular pathomechanisms of the vast majority of DSG2 mutations, however, are unknown. Here, we investigated the homophilic binding of wildtype Dsg2 and two mutations which are associated with ARVC...
October 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29036525/circulating-micrornas-in-arrhythmogenic-right-ventricular-cardiomyopathy-with-ventricular-arrhythmia
#12
Shinya Yamada, Ya-Wen Hsiao, Shih-Lin Chang, Yenn-Jiang Lin, Li-Wei Lo, Fa-Po Chung, Shuo-Ju Chiang, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Chin-Yu Lin, Yao-Ting Chang, Abigail Louise D Te, Yung-Nan Tsai, Shih-Ann Chen
Aims: MicroRNAs (miRNAs) have been implicated in cardiac diseases. This study aimed to characterize the circulating miRNAs in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and correlate the miRNAs with the clinical outcomes of ARVC. Methods and results: This study included 62 patients with ventricular arrhythmia (VA): 28 patients (45%) had definite ARVC, 11 (18%) had borderline or possible ARVC, and 23 (37%) had idiopathic ventricular tachycardia (VT)...
September 25, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28960618/ventricular-tachycardia-ablation-in-arrhythmogenic-right-ventricular-cardiomyopathy-patients-with-tmem43-gene-mutations
#13
Amir Abdelwahab, Martin Gardner, Ratika Parkash, Christopher Gray, John Sapp
INTRODUCTION: Catheter ablation of VT in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is often challenging, frequently requiring multiple or epicardial ablation procedures; TMEM43 gene mutations typically cause aggressive disease. We sought to compare VT ablation outcomes for ARVC patients with and without TMEM43 mutations. METHODS: Patients with prior ablation for ARVC-related VT were reviewed. Demographic, procedural and follow-up data were reviewed retrospectively...
September 27, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28957535/exercise-and-the-right-ventricle-a-potential-achilles-heel
#14
Andre La Gerche, Dhrubo J Rakhit, Guido Claessen
Exercise is associated with unequivocal health benefits and results in many structural and functional changes of the myocardium that enhance performance and prevent heart failure. However, intense exercise also presents a significant hemodynamic challenge in which the right-sided heart chambers are exposed to a disproportionate increase in afterload and wall stress that can manifest as myocardial fatigue or even damage if intense exercise is sustained for prolonged periods. This review focuses on the physiological factors that result in a disproportionate load on the right ventricle during exercise and the long-term consequences...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28940781/right-ventricular-scar-related-ventricular-tachycardia-in-non-ischemic-cardiomyopathy-electrophysiological-characteristics-mapping-and-ablation-of-underlying-heart-disease
#15
Saurabh Kumar, Samuel H Baldinger, Sunil Kapur, Jorge Romero, Nishaki K Mehta, Saagar Mahida, Akira Fujii, Usha B Tedrow, William G Stevenson
BACKGROUND: Right ventricular (RV)-scar related ventricular tachycardia (VT) is often due to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) or cardiac sarcoidosis (CS), but some patients whose clinical course has not been described do not fulfill diagnostic criteria for these diseases. We sought to characterize the electrophysiologic substrate and catheter ablation outcomes of such patients, termed RV cardiomyopathy of unknown source (RCUS). METHODS AND RESULTS: Data of 100 consecutive patients who presented with RV cardiomyopathy and/or RV-related VT for ablation was reviewed (51 ARVC/D, 22 CS; 27 RCUS)...
September 21, 2017: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/28874384/heart-failure-is-common-and-under-recognized-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy-dysplasia
#16
Nisha A Gilotra, Aditya Bhonsale, Cynthia A James, Anneline S J Te Riele, Brittney Murray, Crystal Tichnell, Abhishek Sawant, Chin Siang Ong, Daniel P Judge, Stuart D Russell, Hugh Calkins, Ryan J Tedford
BACKGROUND: Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D. METHODS AND RESULTS: Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. HF was present in 142 patients (49%): 113 had isolated RV involvement and 29 had evidence of LV dysfunction...
September 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28865556/normative-reference-values-of-right-heart-in-competitive-athletes-a-systematic-review-and-meta-analysis
#17
REVIEW
Flavio D'Ascenzi, Antonio Pelliccia, Marco Solari, Pietro Piu, Ferdinando Loiacono, Francesca Anselmi, Stefano Caselli, Marta Focardi, Marco Bonifazi, Sergio Mondillo
Training-induced right ventricular (RV) enlargement is frequent in athletes. Unfortunately, RV dilatation is also a common phenotypic expression and one of the diagnostic criteria of arrhythmogenic RV cardiomyopathy (ARVC). The current echocardiographic reference values derived from the general population can overestimate the presence of RV dilatation in athletes. We performed a meta-analysis of the literature to derive the proper reference values for assessing RV enlargement in competitive athletes. We conducted systematic review of English-language studies in the MEDLINE, Scopus, and Cochrane databases investigating RV size and function by echocardiography and by cardiac magnetic resonance (CMR) in competitive athletes...
September 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28855434/isolated-late-activation-detected-by-magnetocardiography-predicts-future-lethal-ventricular-arrhythmic-events-in-patients-with-arrhythmogenic-right-ventricular-cardiomyopathy
#18
Yoshitaka Kimura, Hiroshi Takaki, Yuko Y Inoue, Yasutaka Oguchi, Tomomi Nagayama, Takahiro Nakashima, Shoji Kawakami, Satoshi Nagase, Takashi Noda, Takeshi Aiba, Wataru Shimizu, Shiro Kamakura, Masaru Sugimachi, Satoshi Yasuda, Hiroaki Shimokawa, Kengo Kusano
BACKGROUND: Risk stratification of ventricular arrhythmias is vital to the optimal management in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We hypothesized that 64-channel magnetocardiography (MCG) would be useful to detect isolated late activation (ILA) by overcoming the limitations of conventional noninvasive predictors of ventricular tachyarrhythmias, including epsilon waves, late potential (LP), and right ventricular ejection fraction (RVEF), in ARVC patients...
August 30, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28843747/arrhythmogenic-right-ventricular-cardiomyopathy-arvc-in-a-young-female-athlete-at-36-weeks-gestation-a-case-report
#19
Hae Yoon Grace Choung, Monika Vyas, Daniel Jacoby, Brian West
A 26year old east African professional athlete presented to the obstetric clinic for a routine visit at 36 weeks gestation. She had a history of Right Ventricular Outflow Tract - Ventricular Tachycardia (RVOT-VT) with an episode of cardiac arrest in the past, and had been treated with ablation 4 years earlier. Her current visit was uneventful, her pregnancy progressing normally. Following the visit she went to a local restaurant where she suffered a cardiac arrest that was unresponsive to therapy. Chest compressions were continued from the time of her collapse until an emergency caesarian section was performed, delivering a healthy female infant...
October 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28818065/case-reports-of-two-pedigrees-with-recessive-arrhythmogenic-right-ventricular-cardiomyopathy-associated-with-homozygous-thr335ala-variant-in-dsg2
#20
Sami Qadri, Olli Anttonen, Juho Viikilä, Eija H Seppälä, Samuel Myllykangas, Tero-Pekka Alastalo, Miia Holmström, Tiina Heliö, Juha W Koskenvuo
BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiac disease, involving changes in ventricular myocardial tissue and leading to fatal arrhythmias. Mutations in desmosomal genes are thought to be the main cause of ARVC. However, the exact molecular genetic etiology of the disease still remains largely inconclusive, and this along with large variabilities in clinical manifestations complicate clinical diagnostics. CASE PRESENTATION: We report two families (n = 20) in which a desmoglein-2 (DSG2) missense variant c...
August 17, 2017: BMC Medical Genetics
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