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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...
November 30, 2016: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
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
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
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...
October 21, 2016: Nephron
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...
March 28, 2016: Annals of Noninvasive Electrocardiology
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
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
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...
October 6, 2016: Journal of Cardiovascular Electrophysiology
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
James Ritchie, Darren Green, Tina Chrysochou, Janet Hegarty, Kelly Handley, Natalie Ives, Keith Wheatley, Graeme Houston, Julian Wright, Ludwig Neyses, Nicholas Chalmers, Patrick Mark, Rajan Patel, Jon Moss, Giles Roditi, David Eadington, Elena Lukaschuk, John Cleland, Philip A Kalra
BACKGROUND: Cardiac abnormalities are frequent in patients with atherosclerotic renovascular disease (ARVD). The Angioplasty and Stenting for Renal Artery Lesions (ASTRAL) trial studied the effect of percutaneous renal revascularization combined with medical therapy compared with medical therapy alone in 806 patients with ARVD. METHODS: This was a pre-specified sub-study of ASTRAL (clinical trials registration, current controlled trials number: ISRCTN59586944), designed to consider the effect of percutaneous renal artery angioplasty and stenting on change in cardiac structure and function, measured using cardiac magnetic resonance (CMR) imaging...
June 2, 2016: Nephrology, Dialysis, Transplantation
Diana Vassallo, Darren Green, James Ritchie, Constantina Chrysochou, James Blunt, Philip A Kalra
BACKGROUND/AIMS: Optimized medical therapy has improved cardiovascular outcomes in the general population. To investigate whether changes in the management of atherosclerotic renovascular disease (ARVD) have had an impact on clinical outcomes. METHODS: Recruitment into this single-center prospective cohort study started in 1986. Data was analyzed retrospectively. Patients were divided into four groups based on relationship of diagnosis year to landmark randomized controlled trials (RCT); group 1 - pre-large RCT data (1986-2000); group 2 - post-early RCT (2001-2004); group 3 - ASTRAL study recruitment era (2004-2009); group 4 - post-ASTRAL (2009-2014)...
2016: Kidney & Blood Pressure Research
Gabriela M Orgeron, Hugh Calkins
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by ventricular arrhythmias, right ventricular dysfunction, and sudden cardiac death. Since the first description of ARVD/C in 1982, there have been major advances in the diagnosis and management of the disease. For instance, the discovery of desmosomal abnormalities as a genetic basis for ARVD/C; the importance of proband status and ventricular ectopy for risk stratification of patients at risk for sudden cardiac death; and the critical role that exercise plays in the development and progression of ARVD/C, just to name a few...
June 2016: Current Cardiology Reports
Fa-Po Chung, Yenn-Jiang Lin, Eric Chong, Shih-Lin Chang, Li-Wei Lo, Yu-Feng Hu, Ta-Chuan Tuan, Tze-Fan Chao, Jo-Nan Liao, Shih-Ann Chen
BACKGROUND: Patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carry the risk of ventricular arrhythmias and sudden cardiac death (SCD). This study investigated the prognostic information of modified moving average T-wave alternans (MMA TWA) in patients with ARVD/C. METHODS: A total of 63 consecutive patients (mean age, 44.7 ± 14.8 years; 38 men) with ARVD/C were enrolled. Baseline characteristics and structural and electrocardiographic parameters were obtained...
February 5, 2016: Canadian Journal of Cardiology
Neda Rastegar, Anneline S J M Te Riele, Cynthia A James, Aditya Bhonsale, Brittney Murray, Crystal Tichnell, Hugh Calkins, Harikrishna Tandri, David A Bluemke, Ihab R Kamel, Stefan L Zimmerman
Purpose To determine the incidence of ventricular fatty replacement and late gadolinium enhancement (LGE) at cardiac magnetic resonance (MR) imaging in patients with arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) and the relationship of these findings to disease severity. Materials and Methods This was a retrospective institutional review board-approved HIPAA-compliant study. All subjects provided written informed consent. Seventy-six patients with ARVD/C were enrolled from 2002 to 2012...
August 2016: Radiology
Pai-Feng Hsu, Hao-Min Cheng, Cheng-Hsueh Wu, Shih-Hsien Sung, Shao-Yuan Chuang, Edward G Lakatta, Frank C P Yin, Pesus Chou, Chen-Huan Chen
BACKGROUND: The prognostic value of the short-term blood pressure variability (BPV) from the 24-hour ambulatory blood pressure monitoring (ABPM) remains controversial. The present study aimed to investigate the long-term prognostic value of a high BPV in normotensive and hypertensive subjects from a community-based population. METHODS: A cohort of 624 normotensive and 633 untreated hypertensive Taiwanese participants (overall 669 men, aged 30-79 years) with baseline ABPM and 20-year all-cause and cardiovascular mortality data was drawn from a community-based survey...
July 2016: American Journal of Hypertension
Cynthia A James
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterised by frequent ventricular arrhythmias and slowly progressive predominant RV dysfunction. Up to two-thirds of ARVD/C patients have mutations in genes encoding the cardiac desmosome. Mutations in other genes are increasingly recognised. Inheritance of ARVD/C is generally autosomal dominant with reduced age-related penetrance and significant variable expressivity. While the full explanation for this phenotypic heterogeneity remains unclear, there is increasing evidence that exercise plays a major role in disease penetrance and arrhythmic risk...
December 2015: Arrhythmia & Electrophysiology Review
James Ritchie, Darren Green, Helen V Alderson, Constantina Chrysochou, Diana Vassallo, Smeeta Sinha, Philip A Kalra
Randomized trials have shown a neutral effect of percutaneous revascularization compared with optimal medical therapy in patients with atherosclerotic renovascular disease (ARVD). However, there are few data to define what constitutes optimal medical therapy. We present a retrospective analysis of 529 ARVD patients. Separate analyses were performed comparing outcomes in patients prescribed/not prescribed beta blocker and antiplatelet agents. Analyses were adjusted for effects of baseline covariates on probability of treatment and on clinical outcome...
February 2016: Journal of the American Society of Hypertension: JASH
Tanyanan Tanawuttiwat, Anneline S J M Te Riele, Binu Philips, Cynthia A James, Brittney Murray, Crystal Tichnell, Abhishek C Sawant, Hugh Calkins, Harikrishna Tandri
BACKGROUND: Epsilon waves and other depolarization abnormalities in the right precordial leads are thought to represent delayed activation of the right ventricular outflow tract in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However, no study has directly correlated cardiac electrical activation with the surface ECG findings in ARVD/C. METHODS AND RESULTS: Thirty ARVD/C patients (mean age 32.7 ± 11.2 years, 16 men) underwent endocardial and epicardial electroanatomical activation mapping in sinus rhythm...
April 2016: Journal of Cardiovascular Electrophysiology
Anneline S J M Te Riele, Cynthia A James, Brittney Murray, Crystal Tichnell, Nuria Amat-Alarcon, Kathleen Burks, Harikrishna Tandri, Hugh Calkins, Michael Polydefkis, Daniel P Judge
Prior reports have identified associations between SCN10A and cardiac disorders, such as atrial fibrillation and Brugada syndrome. We evaluated SCN10A in 151 probands with ARVD/C. In this cohort, 10 putatively pathogenic SCN10A variants were identified, including a novel frameshift insertion. Despite a known role for the encoded protein in peripheral nerve function, the proband with the frameshift variant had no discernible neurological abnormalities. Arrhythmic phenotypes were not different between those with a rare variant in SCN10A and those without...
February 2016: Journal of Cardiovascular Translational Research
Anke R Hodes, Crystal Tichnell, Anneline S J M Te Riele, Brittney Murray, Judith A Groeneweg, Abhishek C Sawant, Stuart D Russell, Karin Y van Spaendonck-Zwarts, Maarten P van den Berg, Arthur A Wilde, Harikrishna Tandri, Daniel P Judge, Richard N W Hauer, Hugh Calkins, J Peter van Tintelen, Cynthia A James
OBJECTIVES: To characterise pregnancy course and outcomes in women with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). METHODS: From a combined Johns Hopkins/Dutch ARVD/C registry, we identified 26 women affected with ARVD/C (by 2010 Task Force Criteria) during 39 singleton pregnancies >13 weeks (1-4 per woman). Cardiac symptoms, treatment and episodes of sustained ventricular arrhythmias (VAs) and heart failure (HF) ≥ Class C were characterised...
February 15, 2016: Heart: Official Journal of the British Cardiac Society
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