keyword
https://read.qxmd.com/read/38411736/prognostic-value-of-right-atrial-strains-in-arrhythmogenic-right-ventricular-cardiomyopathy
#1
JOURNAL ARTICLE
Jin-Yu Zheng, Bing-Hua Chen, Rui Wu, Dong-Aolei An, Ruo-Yang Shi, Chong-Wen Wu, Lang-Lang Tang, Lei Zhao, Lian-Ming Wu
OBJECTIVES: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by progressive fibrofatty infiltration of atrial and ventricular myocardium resulting in adverse cardiac events. Atrial function has been increasingly recognized as prognostically important for cardiovascular disease. As the right atrial (RA) strain is a sensitive parameter to describe RA function, we aimed to analyze the prognostic value of the RA strain in ARVC. METHODS: RA strain parameters were derived from cardiac magnetic resonance (CMR) images of 105 participants with definite ARVC...
February 27, 2024: Insights Into Imaging
https://read.qxmd.com/read/38288614/aav-mediated-delivery-of-plakophilin-2a-arrests-progression-of-arrhythmogenic-right-ventricular-cardiomyopathy-in-murine-hearts-preclinical-evidence-supporting-gene-therapy-in-humans
#2
JOURNAL ARTICLE
Chantal J M van Opbergen, Bitha Narayanan, Chester B Sacramento, Katie M Stiles, Vartika Mishra, Esther Frenk, David Ricks, Grace Chen, Mingliang Zhang, Paul Yarabe, Jonathan Schwartz, Mario Delmar, Chris D Herzog, Marina Cerrone
BACKGROUND: Pathogenic variants in PKP2 (plakophilin-2) cause arrhythmogenic right ventricular cardiomyopathy, a disease characterized by life-threatening arrhythmias and progressive cardiomyopathy leading to heart failure. No effective medical therapy is available to prevent and arrest the disease. We tested the hypothesis that adeno-associated virus vector-mediated delivery of the human PKP2 gene to an adult mammalian heart deficient in PKP2 can arrest disease progression and significantly prolong survival...
January 30, 2024: Circulation. Genomic and Precision Medicine
https://read.qxmd.com/read/38278685/the-heart-has-its-reasons-which-reason-knows-not-a-curious-case-of-chest-pain
#3
JOURNAL ARTICLE
Michael Wallace, Nick Schoenmann, Stanton Royer
BACKGROUND: Electrocardiographic (ECG) findings of T-wave inversions in V1-V3, with or without accompanying epsilon waves, often raise concerns for the rare, but potentially lethal, arrhythmogenic right ventricular cardiomyopathy (ARVC). However, this pattern may be found in pericardial agenesis, an even rarer pathology. Concomitant myocarditis can confuse this presentation further. CASE REPORT: We report a case of a previously healthy man who presented with left-sided chest pain, ECG findings suggestive of ARVC, and a final diagnosis of myocarditis with underlying partial pericardial agenesis...
October 13, 2023: Journal of Emergency Medicine
https://read.qxmd.com/read/38248052/arrhythmogenic-right-ventricular-cardiomyopathy-in-children-a-systematic-review
#4
REVIEW
Stefana Maria Moisa, Elena Lia Spoiala, Eliza Cinteza, Radu Vatasescu, Lacramioara Ionela Butnariu, Crischentian Brinza, Alexandru Burlacu
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We reviewed the literature on diagnosis, risk stratification, and prognosis in the pediatric population with ARVC. In case reports which analyzed children with ARVC, the most common sign was ventricular tachycardia, frequently presenting as dizziness, syncope, or even cardiac arrest...
January 12, 2024: Diagnostics
https://read.qxmd.com/read/38189980/prognostic-value-of-right-ventricular-trabecular-complexity-in-patients-with-arrhythmogenic-cardiomyopathy
#5
JOURNAL ARTICLE
Bing-Hua Chen, Wen-Yi Jiang, Jin-Yu Zheng, Yi-Si Dai, Ruo-Yang Shi, Rui Wu, Dong-Aolei An, Lang-Lang Tang, Jian-Rong Xu, Lei Zhao, Lian-Ming Wu
OBJECTIVES: The present study aimed to investigate the incremental prognostic value of the right ventricular fractal dimension (FD), a novel marker of myocardial trabecular complexity by cardiac magnetic resonance (CMR) in patients with arrhythmogenic cardiomyopathy (ACM). METHODS: Consecutive patients with ACM undergoing CMR were followed up for major cardiac events, including sudden cardiac death, aborted cardiac arrest, and appropriate implantable cardioverter defibrillator intervention...
January 8, 2024: European Radiology
https://read.qxmd.com/read/38113321/utility-of-left-and-right-ventricular-strain-in-arrhythmogenic-right-ventricular-cardiomyopathy-a-prospective-multicenter-registry
#6
MULTICENTER STUDY
Mayooran Namasivayam, Philippe B Bertrand, Samuel Bernard, Timothy W Churchill, Shaan Khurshid, Frank I Marcus, Luisa Mestroni, Jeffrey E Saffitz, Jeffrey A Towbin, Wojciech Zareba, Michael H Picard, Danita Yoerger Sanborn
BACKGROUND: Imaging evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) remains challenging. Myocardial strain assessment by echocardiography is an increasingly utilized technique for detecting subclinical left ventricular (LV) and right ventricular (RV) dysfunction. We aimed to evaluate the diagnostic and prognostic utility of LV and RV strain in ARVC. METHODS: Patients with suspected ARVC (n = 109) from a multicenter registry were clinically phenotyped using the 2010 ARVC Revised Task Force Criteria and underwent baseline strain echocardiography...
December 2023: Circulation. Cardiovascular Imaging
https://read.qxmd.com/read/38075981/case-report-identification-of-novel-cdh2-mutation-p-p183a-het-induced-arrhythmogenic-cardiomyopathy-in-china
#7
Kun Li, Yifei Wang, Jing Yang, Fang Liu, Fulan Liu, Ping Zhang
BACKGROUND: Arrhythmogenic cardiomyopathy/dysplasia (ACM) is an inheritable heart disease closely related to gene variations induced heart fibrofatty replacement, which increases the risk of arrhythmia events and even sudden cardiac death. In this study, we reported a 10-year-old patient with a novel mutation diagnosed with ACM. CASE PRESENTATION: We present the case of a 10-year-old patient admitted with recurrent palpitation, whose electrocardiogram suggested the existence of right ventricle origin premature ventricular contractions and ε wave...
2023: Frontiers in Cardiovascular Medicine
https://read.qxmd.com/read/37979796/basic-and-translational-mechanisms-in-inflammatory-arrhythmogenic-cardiomyopathy
#8
JOURNAL ARTICLE
Morgan Engel, Emily A Shiel, Stephen P Chelko
Arrhythmogenic cardiomyopathy (ACM) is a familial, nonischemic heart disease typically inherited via an autosomal dominant pattern (Nava et al., [1]; Wlodarska et al., [2]). Often affecting the young and athletes, early diagnosis of ACM can be complicated as incomplete penetrance with variable expressivity are common characteristics (Wlodarska et al., [2]; Corrado et al., [3]). That said, of the five desmosomal genes implicated in ACM, pathogenic variants in desmocollin-2 (DSC2) and desmoglein-2 (DSG2) have been discovered in both an autosomal-recessive and autosomal-dominant pattern (Wong et al...
November 17, 2023: International Journal of Cardiology
https://read.qxmd.com/read/37881382/recurrent-syncope-in-a-patient-with-arrhythmogenic-right-ventricular-cardiomyopathy
#9
Efrain Castillo
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an autosomal inherited cardiac condition characterized by fibroadipose tissue replacement of the right ventricular muscle, leading to structural changes and a high risk for ventricular arrhythmias, a gradual decline in right ventricular function, and sudden cardiac death. ARVC has an autosomal dominant inheritance pattern with variable expression among patients, typically affecting young adults. Genetic mutations affecting the cardiac desmosome genes have been widely reported...
September 2023: Curēus
https://read.qxmd.com/read/37850634/homozygous-deletion-of-the-dsg3-terminal-exon-associated-with-acantholytic-blistering-of-the-oral-and-laryngeal-mucosa
#10
Nan Jiang, Taylor B Sewell, Theresa L Kowalski, Aisha Rekab, Susannah Hills, Ladan Fazlollahi, Christine T Lauren, Kimberly Morel, Lakshmi Mehta, Jun Liao
We report a novel homozygous 49.6 kb deletion of chromosome 18q12.1 involving the last exon of DSG3 in dizygotic twins with phenotype consistent with acantholytic blistering of the oral and laryngeal mucosa (ABOLM). The twin siblings presented predominantly with friability of the laryngeal and respiratory mucosa. This is only the second report in the literature of this unusual autosomal recessive blistering disorder. The diagnosis explains the mucosal phenotype of a pemphigus-like disorder without evidence of autoimmune dysfunction...
October 18, 2023: American Journal of Medical Genetics. Part A
https://read.qxmd.com/read/37775650/new-drug-discovery-of-cardiac-anti-arrhythmic-drugs-insights-in-animal-models
#11
JOURNAL ARTICLE
Ashish Kumar Sharma, Shivam Singh, Mehvish Bhat, Kartik Gill, Mohammad Zaid, Sachin Kumar, Anjali Shakya, Junaid Tantray, Divyamol Jose, Rashmi Gupta, Tsering Yangzom, Rajesh Kumar Sharma, Sanjay Kumar Sahu, Gulshan Rathore, Priyanka Chandolia, Mithilesh Singh, Anurag Mishra, Shobhit Raj, Archita Gupta, Mohit Agarwal, Sumaiya Kifayat, Anamika Gupta, Prashant Gupta, Ankit Vashist, Parth Vaibhav, Nancy Kathuria, Vipin Yadav, Ravindra Pal Singh, Arun Garg
Cardiac rhythm regulated by micro-macroscopic structures of heart. Pacemaker abnormalities or disruptions in electrical conduction, lead to arrhythmic disorders may be benign, typical, threatening, ultimately fatal, occurs in clinical practice, patients on digitalis, anaesthesia or acute myocardial infarction. Both traditional and genetic animal models are: In-vitro: Isolated ventricular Myocytes, Guinea pig papillary muscles, Patch-Clamp Experiments, Porcine Atrial Myocytes, Guinea pig ventricular myocytes, Guinea pig papillary muscle: action potential and refractory period, Langendorff technique, Arrhythmia by acetylcholine or potassium...
September 29, 2023: Scientific Reports
https://read.qxmd.com/read/37732886/bilateral-ischemic-stroke-in-a-37-year-old-patient-with-factor-v-leiden-mutation-and-arrhythmogenic-right-ventricular-dysplasia
#12
JOURNAL ARTICLE
Maryla Kuczyńska, Monika Zbroja, Katarzyna Drelich, Olga Pustelniak, Mariusz Kozak, Edyta Opalińska
No abstract text is available yet for this article.
October 26, 2023: Polish Archives of Internal Medicine
https://read.qxmd.com/read/37619879/electroanatomical-voltage-mapping-with-contact-force-sensing-for-diagnosis-of-arrhythmogenic-right-ventricular-cardiomyopathy
#13
JOURNAL ARTICLE
A M Saguner, D Lunk, M Mohsen, Sven Knecht, Deniz Akdis, S Costa, A Gasperetti, F Duru, V A Rossi, C B Brunckhorst
BACKGROUND: Three-dimensional electroanatomical mapping (EAM) can be helpful to diagnose arrhythmogenic right ventricular cardiomyopathy (ARVC). Yet, previous studies utilizing EAM have not systematically used contact-force sensing catheters (CFSC) to characterize the substrate in ARVC, which is the current gold standard to assure adequate tissue contact. OBJECTIVE: To investigate reference values for endocardial right ventricular (RV) EAM as well as substrate characterization in patients with ARVC by using CFSC...
August 22, 2023: International Journal of Cardiology
https://read.qxmd.com/read/37612153/a-case-report-of-triple-organ-transplantation-from-a-donor-after-circulatory-death-using-thoraco-abdominal-normothermic-regional-perfusion
#14
JOURNAL ARTICLE
Hiroshi Kagawa, Matthew Goodwin, Josef Stehlik, Jeffrey Campsen, Talia Baker, Craig H Selzman
Organ transplantation with donation after circulatory death can potentially increase the donor pool. Here, we report the rare case of triple-organ (heart/liver/kidney) transplantation from a donor after circulatory death using thoraco-abdominal normothermic regional perfusion. The recipient was a 61-year-old man with end-stage heart failure, liver failure, and kidney failure secondary to arrhythmogenic right ventricular dysplasia. He received a heart/liver/kidney transplantation from a donor after circulatory death...
August 21, 2023: Transplantation Proceedings
https://read.qxmd.com/read/37433658/management-of-arrhythmogenic-right-ventricular-cardiomyopathy
#15
REVIEW
Sayed Al-Aidarous, Alexandros Protonotarios, Perry M Elliott, Pier D Lambiase
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease characterised by fibrofatty replacement of the ventricular myocardium due to specific mutations, leading to ventricular arrhythmias and sudden cardiac death. Treating this condition can be challenging due to progressive fibrosis, phenotypic variations and small patient cohorts limiting the feasibility of conducting meaningful clinical trials. Although widely used, the evidence base for anti-arrhythmic drugs is limited. Beta-blockers are theoretically sound, yet their efficacy in reducing arrhythmic risk is not robust...
July 11, 2023: Heart
https://read.qxmd.com/read/37394208/some-topical-aspects-of-the-sports-heart-problem-literature-review-part-2
#16
REVIEW
A Korotkikh, Yu Vakhnenko, M Kashtanov
Electrocardiography occupies a special place among a significant list of other methods for diagnosing the pathology of the cardiovascular system of athletes. Often its results differ significantly from those in the general population, being a consequence of the adaptation of the heart to economical functioning at rest and super-intensive work in training and competitions. This review focuses on the features of the "athlete's electrocardiogram (ECG)". In particular, those changes that are not a reason for removing athletes from physical activity, but in combination with known factors can lead to more serious changes up to sudden cardiac death...
June 30, 2023: Current Problems in Cardiology
https://read.qxmd.com/read/37284396/non-sustained-ventricular-tachycardia-as-a-presentation-of-arrhythmogenic-right-ventricular-cardiomyopathy
#17
Adina Amin, Nadian Bailey, Amanda Warren, Bharath Reddy
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare disorder with familial (autosomal dominant) predisposition and can be challenging to diagnose. Non-sustained ventricular tachycardia (NSVT) is a relatively uncommon and short-lived arrhythmia when seen in the general, healthy population. NSVT with a left bundle branch block morphology is usually idiopathic but may also be seen in ARVC. It can also be associated with poorer prognosis and increased mortality. Repetitive monomorphic ventricular ectopic beats may suggest ARVC, but could also be idiopathic...
May 2023: Curēus
https://read.qxmd.com/read/37246080/genetic-profile-and-genotype-phenotype-correlations-in-childhood-cardiomyopathy
#18
JOURNAL ARTICLE
Chloé Wanert, Fedoua El Louali, Sarab Al Dybiat, Karine Nguyen, Stéphane Zaffran, Caroline Ovaert
BACKGROUND: Genetic cardiomyopathy is a rare disease in childhood. AIMS: To analyse clinical and genetic aspects of a paediatric cardiomyopathy population, and to establish genotype-phenotype correlations. METHODS: We performed a retrospective study of all patients with idiopathic cardiomyopathy aged<18years in Southeast France. Secondary causes of cardiomyopathy were excluded. All data (clinical, echocardiography, genetic testing) were collected retrospectively...
2023: Archives of Cardiovascular Diseases
https://read.qxmd.com/read/36977546/implantable-cardioverter-defibrillator-shocks-during-long-term-follow-up-in-arrhythmogenic-right-ventricular-cardiomyopathy
#19
EDITORIAL
Steven A Muller, Anneline S J M Te Riele
No abstract text is available yet for this article.
March 28, 2023: Heart
https://read.qxmd.com/read/36914249/exercise-and-arrhythmogenic-cardiomyopathy-all-things-in-moderation
#20
EDITORIAL
Graham Bevan, Babak Nazer
No abstract text is available yet for this article.
March 13, 2023: Heart
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