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https://www.readbyqxmd.com/read/28214153/arrhythmogenic-right-ventricular-dysplasia-atypical-clinical-presentation
#1
José Marçalo, Luiz Menezes Falcão
A 67-year-old man was admitted to our hospital after episodes of syncope preceded by malaise and diffuse neck and chest discomfort. No family history of cardiac disease was reported. Laboratory workup was within normal limits, including D-dimers, serum troponin I and arterial blood gases. The electrocardiogram showed sinus rhythm with T-wave inversion in leads V1 to V3. Computed tomography angiography to investigate pulmonary embolism showed no abnormal findings. Transthoracic echocardiography (TTE) displayed massive enlargement of the right ventricle with intact interatrial septum and no pulmonary hypertension...
February 14, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/28167090/ablation-of-ventricular-tachycardia-in-arrhythmogenic-right-ventricular-dysplasia
#2
REVIEW
Rajeev K Pathak, Fermin C Garcia
Endocardial and epicardial electroanatomical mapping and ablation is a safe and effective therapy in the treatment of right ventricle arrhythmias occurring in the setting of arrhythmogenic right ventricular cardiomyopathy (ARVD). Careful mapping and ablation plans must be tailored for each patient based on comorbidities and ventricular tachycardia morphologies. This review focuses on the catheter ablation for ventricular arrhythmias in patients with ARVD.
March 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28131707/the-application-of-signal-average-ecg-in-the-prediction-of-recurrences-after-catheter-ablation-of-ventricular-arrhythmias-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#3
Ying-Chieh Liao, Fa-Po Chung, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Chin-Yu Lin, Yao-Ting Chang, Ming-Hsiung Hsieh, Shih-Ann Chen
BACKGROUND: The changes of signal averaged ECG (SAECG) in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) undergoing radiofrequency catheter ablation (RFCA) of ventricular arrhythmias (VAs) remains unknown. METHODS: Between 2010 and 2014, a total of 81 ARVD/C patients underwent endocardial and/or epicardial RFCA for drug-refractory VAs. Seventy patients (mean age 46.2±14.1years, 37 males) achieving acute procedure success (negative inducibility) were enrolled...
January 18, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28097316/evaluation-of-structural-progression-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#4
Thomas P Mast, Cynthia A James, Hugh Calkins, Arco J Teske, Crystal Tichnell, Brittney Murray, Peter Loh, Stuart D Russell, Birgitta K Velthuis, Daniel P Judge, Dennis Dooijes, Ryan J Tedford, Jeroen F van der Heijden, Harikrishna Tandri, Richard N Hauer, Theodore P Abraham, Pieter A Doevendans, Anneline S J M Te Riele, Maarten J Cramer
Importance: Considerable research has described the arrhythmic course of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However, objective data characterizing structural progression, such as ventricular enlargement and cardiac dysfunction, in ARVD/C are relatively scarce. Objectives: To define the extent of structural progression, identify determinants of structural progression, and determine the association between structural progression and electrocardiographic (ECG) changes in patients with ARVD/C...
January 11, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28096150/hemodynamic-determinants-of-the-short-term-blood-pressure-variability-differential-roles-of-arterial-stiffness-and-wave-reflection
#5
Pai-Feng Hsu, Hao-Min Cheng, Shih-Hsien Sung, Shao-Yuan Chuang, Edward G Lakatta, Frank C P Yin, Pesus Chou, Chen-Huan Chen
BACKGROUND: A high 24-hour ambulatory diastolic (DBP) but not systolic (SBP) blood pressure variability (BPV) is significantly predictive of long-term cardiovascular mortality in untreated hypertensive subjects, independent of office or 24-hour SBP. The present study was aimed to investigate hemodynamic factors that are independently associated with systolic and diastolic BPV from the 24-hour ambulatory blood pressure monitoring (ABPM). METHODS: A cohort of 624 normotensive and 633 untreated hypertensive participants with baseline ABPM was drawn from a community-based survey...
January 17, 2017: American Journal of Hypertension
https://www.readbyqxmd.com/read/28087964/arrythmogenic-right-ventricular-dysplasia
#6
M K Anis Munirah, W K Lim, I M Sharifah Ainon
A 10-year-old well and asymptomatic female was referred for screening of acute right ventricular dilatation (ARVD) as she had an elder brother diagnosed with ARVD whom died of sudden cardiac death. Electrocardiography (ECG), transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) were performed. Results of these investigations were suggestive of ARVD. Despite being a rare cardiac disease and largely unrecognised in children and young adult population, ARVD is an important cause of ventricular arrhythmias in this group of patients and is one of the causes of sudden cardiac death (SCD) in this population...
December 2016: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28069705/multilevel-analyses-of-scn5a-mutations-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-suggest-non-canonical-mechanisms-for-disease-pathogenesis
#7
Anneline S J M Te Riele, Esperanza Agullo-Pascual, Cynthia A James, Alejandra Leo-Macias, Marina Cerrone, Mingliang Zhang, Xianming Lin, Bin Lin, Nara L Sobreira, Nuria Amat-Alarcon, Roos F Marsman, Brittney Murray, Crystal Tichnell, Jeroen F van der Heijden, Dennis Dooijes, Toon A B van Veen, Harikrishna Tandri, Steven J Fowler, Richard N W Hauer, Gordon Tomaselli, Maarten P van den Berg, Matthew R G Taylor, Francesca Brun, Gianfranco Sinagra, Arthur A M Wilde, Luisa Mestroni, Connie R Bezzina, Hugh Calkins, J Peter van Tintelen, Lei Bu, Mario Delmar, Daniel P Judge
AIMS: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is often associated with desmosomal mutations. Recent studies suggest an interaction between the desmosome and sodium channel protein Nav1.5. We aimed to determine the prevalence and biophysical properties of mutations in SCN5A (the gene encoding Nav1.5) in ARVD/C. METHODS AND RESULTS: We performed whole-exome sequencing in six ARVD/C patients (33% male, 38.2 ± 12.1 years) without a desmosomal mutation...
January 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28062531/epsilon-wave-on-an-electronic-loop-in-a-case-of-arrhythmogenic-right-ventricular-dysplasia-with-myocarditis-an-updated-definition-of-the-epsilon-wave
#8
Guy Hugues Fontaine, Guillaume Duthoit, Guoliang Li, L Andreoletti, Estelle Gandjbakhch, Robert Frank
A young man presented with a history of myocarditis with palpitations and dizziness. He had implantation of a loop recorder that showed repetitive short episodes of VT. In addition, there were fragmented potentials immediately following the large and sharp electrograms (EGMs) before as well as after episodes of VT suggesting an Epsilon wave. This signal can be observed in multiple cardiac conditions including coronary artery disease. It was originally recorded on the epicardium as well as on the endocardium...
January 6, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28008980/antiretroviral-treatment-with-efavirenz-disrupts-the-blood-brain-barrier-integrity-and-increases-stroke-severity
#9
Luc Bertrand, Levi Dygert, Michal Toborek
The introduction of antiretroviral drugs (ARVd) changed the prognosis of HIV infection from a deadly disease to a chronic disease. However, even with undetectable viral loads, patients still develop a wide range of pathologies, including cerebrovascular complications and stroke. It is hypothesized that toxic side effects of ARVd may contribute to these effects. To address this notion, we evaluated the impact of several non-nucleoside reverse transcriptase inhibitors (NNRTI; Efavirenz, Etravirine, Rilpivirine and Nevirapine) on the integrity of the blood-brain barrier, and their impact on severity of stroke...
December 23, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27932238/gender-differences-in-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-clinical-manifestations-electrophysiological-properties-substrate-characteristics-and-prognosis-of-radiofrequency-catheter-ablation
#10
Chin-Yu Lin, Fa-Po Chung, Yenn-Jiang Lin, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Yao-Ting Chang, Yun-Yu Chen, Rohit Walia, Abigail Louise D Te, Shinya Yamada, Shih-Ann Chen
BACKGROUND: Gender differences in the penetrance and clinical expression of genetic mutations have been reported in patients with arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C). Our study aimed at clarifying the impact of gender on ventricular substrates and clinical outcomes after radiofrequency catheter ablation (RFCA). METHODS: Patients with ARVD/C underwent RFCA for drug-refractory ventricular arrhythmias (VAs) were consecutively enrolled...
January 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/27927187/the-importance-of-proteinuria-and-prior-cardiovascular-disease-in-all-major-clinical-outcomes-of-atherosclerotic-renovascular-disease-a-single-center-observational-study
#11
Diana Vassallo, James Ritchie, Darren Green, Constantina Chrysochou, Joseph Blunt, Philip A Kalra
BACKGROUND: Identification of patients at risk of developing adverse events would enable aggressive medical therapy and possibly targeted revascularization. The aim of this study is to characterize the determinants of long-term outcomes in atherosclerotic renovascular disease (ARVD). METHODS: Patients with a radiological diagnosis of ARVD were recruited into this single-center prospective cohort study between 1986 and 2014. Data collected included baseline co-morbid conditions, annualized prescribed medications and laboratory data (serum creatinine [υmol/L], proteinuria [g/24 h])...
December 7, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27900527/should-all-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-undergo-epicardial-catheter-ablation
#12
Andreas Müssigbrodt, Elena Efimova, Helge Knopp, Livio Bertagnolli, Nikolaos Dagres, Sergio Richter, Daniela Husser, Andreas Bollmann, Gerhard Hindricks, Arash Arya
BACKGROUND: Epicardial radiofrequency catheter ablation is currently considered as the therapeutic option of choice in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and recurrent ventricular tachycardia (VT). We hypothesised that inducibility of VT may guide the ablation strategy and thereby affect long-term results. Additional epicardial ablation was only performed if VT were still inducible after thorough endocardial ablation. METHODS: The objective was to examine an inducibility-guided ablation approach by comparing the long-term results between endocardial and epi-endocardial radiofrequency catheter ablation of VT in a large cohort of patients with ARVD/C...
March 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/27871237/a-unique-association-of-arrhythmogenic-right-ventricular-dysplasia-and-acute-myocarditis-as-assessed-by-cardiac-mri-a-case-report
#13
Andrea Ponsiglione, Marta Puglia, Carmine Morisco, Luigi Barbuto, Antonio Rapacciuolo, Mario Santoro, Letizia Spinelli, Bruno Trimarco, Alberto Cuocolo, Massimo Imbriaco
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD), is a genetic disorder of the heart, which mainly involves the right ventricle. It is characterized by hypokinetic areas at the free wall of the right ventricle (RV) or both ventricles, where myocardium is replaced by fibrous or fatty tissue. ARVD is an important cause of ventricular arrhythmias in children and young adults. Although the transmission of the disease is based on hereditary, in young adults it may not show any symptoms...
November 21, 2016: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/27790050/arrhythmogenic-right-ventricular-dysplasia-in-neuromuscular-disorders
#14
REVIEW
Josef Finsterer, Claudia Stöllberger
OBJECTIVES: Arrhythmogenic right ventricular dysplasia (ARVD) is a rare, genetic disorder predominantly affecting the right ventricle. There is increasing evidence that in some cases, ARVD is due to mutations in genes, which have also been implicated in primary myopathies. This review gives an overview about myopathy-associated ARVD and how these patients can be managed. METHODS: A literature review was done using appropriate search terms. RESULTS: The myopathy, which is most frequently associated with ARVD, is the myofibrillar myopathy due to desmin mutations...
2016: Clinical Medicine Insights. Cardiology
https://www.readbyqxmd.com/read/27764832/attenuation-of-renal-functional-decline-following-angioplasty-and-stenting-in-atherosclerotic-renovascular-disease
#15
Rodrigo Hagemann, Vanessa Dos Santos Silva, Fábio Cardoso Carvalho, Pasqual Barretti, Luis Cuadrado Martin, Diana Vassallo, Philip A Kalra, Roberto Jorge da Silva Franco
BACKGROUND: To date, renal revascularization has not been shown to be advantageous when compared to optimized medical treatment in patients with atheromatous renovascular disease (ARVD). This study aims to investigate the effect of revascularization in patients with pre-intervention worsening renal function and in those with stable renal function. PATIENTS AND METHODS: In this single-centre observational study, patients who were diagnosed with at least 60% angiographic stenosis unilaterally or bilaterally between January 1996 and October 2008 and who were followed-up until February 2011 were retrospectively analysed...
2017: Nephron
https://www.readbyqxmd.com/read/27610610/evaluation-of-tp-e-interval-tp-e-qt-ratio-and-tp-e-qtc-ratio-in-patients-with-asymptomatic-arrhythmogenic-right-ventricular-cardiomyopathy
#16
Elnur Alizade, Mahmut Yesin, Mehmet Vefik Yazicioğlu, Ekrem Bilal Karaayvaz, Adem Atici, Şükrü Arslan, Anıl Avci, Göksel Acar, Mustafa Tabakci, Servet Izci, Selçuk Pala
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by progressive replacement of ventricular myocytes with variable amounts of fibrous and adipose tissue. Several studies have suggested that the interval from the peak to the end of the electrocardiographic T wave (Tp-e) may correspond to the transmural dispersion of repolarization and that increased Tp-e interval and Tp-e/QT ratio are associated with malignant ventricular arrhythmias. The aim of this study was to evaluate repolarization dispersion measured from the 12-lead surface electrocardiogram (including Tp-e interval, Tp-e/QT, and Tp-e/QTc ratio) in asymptomatic ARVD patients METHODS: We selected 27 patients with asymptomatic ARVD and 27 age- and gender-match young, healthy volunteers...
January 2017: Annals of Noninvasive Electrocardiology
https://www.readbyqxmd.com/read/27599902/utility-of-speckle-tracking-echocardiography-imaging-in-patients-with-asymptomatic-and-symptomatic-arrhythmogenic-right-ventricular-cardiomyopathy
#17
Elnur Alizade, Mahmut Yesin, Mehmet Mustafa Tabakci, Anıl Avci, Mustafa Bulut, Göksel Acar, Zeki Şimşek, Servet Izci, Süleyman Barutçu, Selçuk Pala
BACKGROUND: Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by the progressive replacement of ventricular myocytes with variable amounts of fibrous and adipose tissue. Several studies have suggested that speckle tracking echocardiographic (STE) parameters such as strain (S) and strain rate (SR) may prove useful in the early detection of right ventricular (RV) dysfunction. Therefore, the aim of this study was to evaluate RV myocardial function using the STE method in both asymptomatic and symptomatic patients with ARVD and to assess its potential role in the differential diagnosis of these two presentations...
November 2016: Echocardiography
https://www.readbyqxmd.com/read/27580740/clinical-characterisation-and-risk-stratification-of-patients-with-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy-%C3%A2-50%C3%A2-years-of-age
#18
M J van der Pols, T P Mast, P Loh, J F van der Heijden, M J Cramer, R N Hauer, A S J M Te Riele
PURPOSE: With the increased use of genetic testing for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), this disease is being increasingly recognised among elderly patients. However, elderly ARVD/C patients were underrepresented in prior cohorts. We aimed to describe the phenotypical characteristics and outcomes among ARVD/C patients surviving ≥50 years. METHODS: We assessed detailed phenotypical data of 29 patients who (1) presented at ≥50 years of age; and (2) fulfilled 2010 Task Force Criteria (TFC) for ARVD/C by last follow-up...
December 2016: Netherlands Heart Journal
https://www.readbyqxmd.com/read/27572111/influence-of-genotype-on-structural-atrial-abnormalities-and-atrial-fibrillation-or-flutter-in-arrhythmogenic-right-ventricular-dysplasia-cardiomyopathy
#19
Mimount Bourfiss, Anneline S J M Te Riele, Thomas P Mast, Maarten J Cramer, Jeroen F VAN DER Heijden, Toon A B VAN Veen, Peter Loh, Dennis Dooijes, Richard N W Hauer, Birgitta K Velthuis
INTRODUCTION: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is associated with desmosomal mutations. Although desmosomal disruption affects both ventricles and atria, little is known about atrial involvement in ARVD/C. OBJECTIVE: To describe the extent and clinical significance of structural atrial involvement and atrial arrhythmias (AA) in ARVD/C stratified by genotype. METHODS: We included 71 patients who met ARVD/C Task Force Criteria and underwent cardiac magnetic resonance (CMR) imaging and molecular genetic analysis...
December 2016: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/27398195/right-ventricular-thrombus-formation-in-a-patient-with-arrhythmogenic-right-ventricular-dysplasia-following-radiofrequency-ablation
#20
Stephan Kemmner, Hasema Lesevic, Tilko Reents, Heribert Schunkert, Christof Burgdorf
A middle-aged female suffering from ARVD presented for routine follow-up 8 weeks after right ventricular radiofrequency ablation of recurring ventricular tachycardia. Echocardiography revealed two right ventricular thrombi in the scar area of right ventricular radiofrequency ablation. Ablation-related thromboembolic events should be considered as possible complication in patients suffering from ARVD.
June 2016: Clinical Case Reports
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