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arrhythmogenic right ventricular cardiomyopathy

Maciej Kubala, Rajeev K Pathak, Shuanglun Xie, Ruben Casado Arroyo, Cory M Tschabrunn, Tatsuya Hayashi, Fermin C Garcia, Gregory E Supple, Pasquale Santangeli, David S Frankel, Erica S Zado, David J Callans, Francis E Marchlinski
BACKGROUND: Repolarization abnormalities in arrhythmogenic right ventricular (RV) cardiomyopathy and their relationship to ventricular tachycardia substrate are incompletely understood. METHODS AND RESULTS: In 40 patients (29 men, mean age 38 years) with arrhythmogenic RV cardiomyopathy, we compared the extent and location of abnormal T (NegT) waves ≥1 mm in depth (n=32) and downsloping elevated ST segment (n=13), in ≥2 adjacent leads, to area and location of endocardial bipolar (<1...
March 2018: Circulation. Arrhythmia and Electrophysiology
Adalena Tsatsopoulou
No abstract text is available yet for this article.
May 1, 2018: International Journal of Cardiology
E Gandjbakhch, E Varlet, G Duthoit, V Fressart, P Charron, C Himbert, C Maupain, C Bordet, F Hidden-Lucet, J Nizard
INTRODUCTION: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children. METHODS: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry. Cardiac and obstetrical events having occurred during pregnancy/delivery/post-partum periods and neonatal data/follow-up were collected...
May 1, 2018: International Journal of Cardiology
Patrícia B S Celestino-Soper, Ty C Lynnes, Lili Zhang, Karen Ouyang, Samuel Wann, Victoria L Clyde, Matteo Vatta
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder that may lead to sudden death and can affect humans and other primates. In 2012, the alpha male bonobo of the Milwaukee County Zoo died suddenly and histologic evaluation found features of ARVC. This study sought to discover a possible genetic cause for ARVC in this individual. We sequenced our subject's DNA to search for deleterious variants in genes involved in cardiovascular disorders. Variants found were annotated according to the human genome, following currently available classification used for human diseases...
March 12, 2018: Scientific Reports
Mangyuan Wang, Kai Chen, Xiao Chen, Liang Chen, Jiangping Song, ShengShou Hu
Arrhythmogenic right ventricular cardiomyopathy (ARVC) and dilated cardiomyopathy (DCM), despite being two dramatically different entities, have overlapping phenotypes. As it is easy to misdiagnose between ARVC and DCM, there is a need to establish a new differential diagnostic parameter to differentiate the two. We investigated the utility of endomyocardial biopsy (EMB) for the differential diagnosis, and our study had three aims. The first was to verify the EMB high diagnostic efficacy. The second was to investigate the EMB perforation risk at the right ventricle (RV) free wall of end-stage ARVC...
February 14, 2018: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Prabhpreet Singh, Amit Noheria
Invasive electrophysiology (EP) mapping and catheter ablation has increasingly become the standard of care for many cardiac arrhythmias like supraventricular tachycardias, atrial fibrillation, premature ventricular complexes (PVC), and monomorphic ventricular tachycardia. In this review, we discuss the recent progress made in the mapping and ablation of ventricular fibrillation (VF). Ventricular activation during VF is apparently disorganized, making mapping and interpretation difficult. Prolonged mapping during VF would require mechanical circulatory support as VF causes complete hemodynamic collapse...
March 6, 2018: Current Treatment Options in Cardiovascular Medicine
Adalena Tsatsopoulou, Eduardo Bossone
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is the most common phenotype described within the spectrum of arrhythmogenic cardiomyopathies. It usually presents in early adolescence with severe ventricular arrhythmias along with cardiac structural and functional alterations mainly of the right ventricular myocardium. Though the estimated prevalence of ARVC in the general population is only 1:5000, it represents one of the most common causes of juvenile sudden death. However, detection of early RV dysfunction in ARVC may be challenging requiring high clinical suspicion and an algorithmic approach...
April 15, 2018: International Journal of Cardiology
Giuseppe Cannavale, Marco Francone, Nicola Galea, Francesco Vullo, Antonio Molisso, Iacopo Carbone, Carlo Catalano
Ectopic cardiac fatty images are not rarely detected incidentally by computed tomography and cardiac magnetic resonance, or by exams focused on the heart as in general thoracic imaging evaluations. A correct interpretation of these findings is essential in order to recognize their normal or pathological meaning, focusing on the eventually associated clinical implications. The development of techniques such as computed tomography and cardiac magnetic resonance allowed a detailed detection and evaluation of adipose tissue within the heart...
2018: BioMed Research International
Shuanglun Xie, Benoit Desjardins, Maciej Kubala, Jackson Liang, Jiandu Yang, Rob J van der Geest, Robert Schaller, Michael Riley, David Callans, Erica Zado Pac, Francis Marchlinski, Saman Nazarian
BACKGROUND: Criteria for identification of anatomic ventricular tachycardia (VT) substrates in arrhythmogenic right ventricular cardiomyopathy (ARVC) on late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) are unclear. OBJECTIVE: We sought to define a) the association of regional RV epicardial voltage amplitude with the distribution of LGE, and b) appropriate image signal intensity (SI) thresholds for VT substrate identification, in ARVC. METHODS: Pre-procedural LGE-CMR and epicardial electrogram mapping were performed in 10 ARVC patients...
March 1, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
C M Mak, S Pl Chen, N S Mok, W K Siu, H Hc Lee, C K Ching, P T Tsui, N C Fong, Y P Yuen, W T Poon, C Y Law, Y K Chong, Y W Chan, T C Yung, K Yy Fan, C W Lam
INTRODUCTION: Hereditary channelopathies and cardiomyopathies are potentially lethal and are clinically and genetically heterogeneous, involving at least 90 genes. Genetic testing can provide an accurate diagnosis, guide treatment, and enable cascade screening. The genetic basis among the Hong Kong Chinese population is largely unknown. We aimed to report on 28 unrelated patients with positive genetic findings detected from January 2006 to December 2015. METHODS: Sanger sequencing was performed for 28 unrelated patients with a clinical diagnosis of channelopathies or cardiomyopathies, testing for the following genes: KCNQ1, KCNH2, KCNE1, KCNE2, and SCN5A, for long QT syndrome; SCN5A for Brugada syndrome; RYR2 for catecholaminergic polymorphic ventricular tachycardia; MYH7 and MYBPC3 for hypertrophic cardiomyopathy; LMNA for dilated cardiomyopathy; and PKP2 and DSP for arrhythmogenic right ventricular dysplasia/cardiomyopathy...
March 2, 2018: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
Kathleen T Hickey, Amir Elzomor
The discovery of the human genome has ushered in a new era of molecular testing, advancing our knowledge and ability to identify cardiac channelopathies. Genetic variations can affect the opening and closing of the potassium, sodium, and calcium channels, resulting in arrhythmias and sudden death. Cardiac arrhythmias caused by disorders of ion channels are known as cardiac channelopathies. Nurses are important members of many interdisciplinary teams and must have a general understanding of the pathophysiology of the most commonly encountered cardiac channelopathies, electrocardiogram characteristics, approaches to treatment, and care for patients and their families...
2018: AACN Advanced Critical Care
Thijs H Oude Munnink, Anna Demmer, Roel H J Slenter, Kris L L Movig
The authors present a case of a 69-year-old man with arrhythmogenic right ventricular cardiomyopathy controlled with amiodarone and an infected orthopedic prosthesis requiring treatment with rifampicin. This combination involves a pharmacokinetic drug-drug interaction leading to subtherapeutic drug concentrations of amiodarone and its active metabolite. The long half-life of amiodarone and its active metabolite in combination with the late onset and offset of cytochrome P4503A (CYP3A4) induction by rifampicin makes this a challenging drug-drug interaction to cope with in clinical practice...
February 20, 2018: Therapeutic Drug Monitoring
Cristina Mondello, Elvira Ventura Spagnolo, Luigi Cardia, Orazio Ventura Spagnolo, Patrizia Gualniera, Antonina Argo
The most common cause of sudden death during sexual intercourse in adults is heart disease, and it is usually the male, whereas the death of the woman is unusual. Generally, in these cases, death occurs as a result of cardiovascular disease. The authors report an unusual case of the sudden death of a young woman during sexual intercourse. The post-mortem investigations (autopsy, cardiac nuclear magnetic resonance and cardiac histology) demonstrated a previously undiagnosed arrhythmogenic right ventricular cardiomyopathy...
January 1, 2018: Medico-legal Journal
Li Wang, Shenghua Liu, Hongliang Zhang, Shengshou Hu, Yingjie Wei
Arrhythmogenic cardiomyopathy (AC) is an inherited disorder that is predominantly present in the right ventricular myocardium. Mutations in the genes encoding the desmosomal protein are thought to underlie the pathogenesis of AC. Since AC is genetically heterogeneous and phenotypically diverse, modifier genes and environmental factors have an important role in disease expression. The aim of the present study was to identify AC-associated desmosomal gene variations, and examine the expression levels of intercalated disc proteins in AC patients who carry the variations (DSG2 p...
March 2018: Experimental and Therapeutic Medicine
Julia H Indik
No abstract text is available yet for this article.
February 2018: Circulation. Arrhythmia and Electrophysiology
Gabriela M Orgeron, Anneline Te Riele, Crystal Tichnell, Weijia Wang, Brittney Murray, Aditya Bhonsale, Daniel P Judge, Ihab R Kamel, Stephan L Zimmerman, Harikrishna Tandri, Hugh Calkins, Cynthia A James
BACKGROUND: Ventricular arrhythmias are a feared complication of arrhythmogenic right ventricular dysplasia/cardiomyopathy. In 2015, an International Task Force Consensus Statement proposed a risk stratification algorithm for implantable cardioverter-defibrillator placement in arrhythmogenic right ventricular dysplasia/cardiomyopathy. METHODS AND RESULTS: To evaluate performance of the algorithm, 365 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients were classified as having a Class I, IIa, IIb, or III indication per the algorithm at baseline...
February 2018: Circulation. Arrhythmia and Electrophysiology
Jay Brieler, Matthew A Breeden, Jane Tucker
The definition and classification of cardiomyopathy have evolved considerably in recent years. Cardiomyopathy can be separated into primary (genetic, mixed, or acquired) and secondary categories, which result in varied phenotypes including dilated, hypertrophic, and restrictive patterns. Hypertrophic cardiomyopathy is the most common primary cardiomyopathy and can cause exertional dyspnea, presyncope, atypical chest pain, heart failure, and sudden cardiac death. Dilated cardiomyopathy can be genetic or acquired and typically presents with classic symptoms of heart failure with reduced ejection fraction...
November 15, 2017: American Family Physician
Laurens P Bosman, Arjan Sammani, Cynthia A James, Julia Cadrin-Tourigny, Hugh Calkins, J Peter van Tintelen, Richard N W Hauer, Folkert W Asselbergs, Anneline S J M Te Riele
While many studies evaluate predictors for ventricular arrhythmias in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), a systematic review consolidating this evidence is currently lacking. Therefore, we searched MEDLINE and Embase for studies analyzing predictors for ventricular arrhythmias (sustained ventricular tachycardia/fibrillation (VT/VF), appropriate implantable cardioverter-defibrillator therapy, or sudden cardiac death) in definite ARVC patients, borderline ARVC patients, and ARVC-associated mutation carriers...
February 2, 2018: Heart Rhythm: the Official Journal of the Heart Rhythm Society
A Denis, F Sacher, N Derval, R Martin, H S Lim, T Pambrun, G Massoullie, J Duchateau, H Cochet, X Pillois, G Cheniti, A Frontera, M Takigawa, K Vlachos, C Martin, T Kitamura, M Hocini, H Douard, P Jaïs, M Haïssaguerre
Aims: To compare the arrhythmic response to isoproterenol and exercise testing in newly diagnosed arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. Methods and results: We studied isoproterenol [continuous infusion (45 µg/min) for 3 min] and exercise testing (workload increased by 30 W every 3 min) performed in consecutive newly diagnosed ARVC patients. Both tests were evaluated with regard to the incidence of (i) polymorphic premature ventricular contractions (PVCs) and couplet(s) or (ii) sustained or non-sustained ventricular tachycardia (VT) with left bundle branch block [excluding right ventricular outflow tract VT]; and compared to a control group referred for the evaluation of PVCs without structural heart disease...
February 1, 2018: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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
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