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Elijah R. Behr

Bio: Elijah R. Behr is an academic researcher from St George’s University Hospitals NHS Foundation Trust. The author has contributed to research in topics: Brugada syndrome & Sudden cardiac death. The author has an hindex of 52, co-authored 274 publications receiving 11337 citations. Previous affiliations of Elijah R. Behr include University of London & St. George's University.


Papers
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Journal ArticleDOI
01 Oct 2013-Europace
TL;DR: This international consensus statement is the collaborative effort of three medical societies representing electrophysiology in North America, Europe, and Asian-Pacific area and summarizes the opinion of the international writing group members based on their own experience and on a general review of the literature.
Abstract: This international consensus statement is the collaborative effort of three medical societies representing electrophysiology in North America, Europe, and Asian-Pacific area: the Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA), and the Asia Pacific Heart Rhythm Society. The objective of the consensus document is to provide clinical guidance for diagnosis, risk stratification, and management of patients affected by inherited primary arrhythmia syndromes. It summarizes the opinion of the international writing group members based on their own experience and on a general review of the literature with respect to the clinical data on patients affected by channelopathies. This document does not address the indications of genetic testing in patients affected by inherited arrhythmias and their family members. Diagnostic, prognostic, and therapeutic implications of the results of genetic testing are also not included in this document because this topic has been covered by a recent publication1 coauthored by some of the contributors of this consensus document, and it remains the reference text on this topic. Guidance for the evaluation of patients with idiopathic ventricular fibrillation, sudden arrhythmic death …

555 citations

Journal ArticleDOI
Connie R. Bezzina1, Julien Barc1, Yuka Mizusawa1, Carol Ann Remme1, Jean-Baptiste Gourraud, Floriane Simonet2, Floriane Simonet3, Floriane Simonet4, Arie O. Verkerk1, Peter J. Schwartz, Lia Crotti5, Federica Dagradi5, Pascale Guicheney6, Pascale Guicheney2, Véronique Fressart2, Véronique Fressart6, Antoine Leenhardt7, Antoine Leenhardt2, Charles Antzelevitch, Susan Bartkowiak, Martin Borggrefe8, Rainer Schimpf8, Eric Schulze-Bahr, Sven Zumhagen, Elijah R. Behr9, Rachel Bastiaenen9, Jacob Tfelt-Hansen10, Jacob Tfelt-Hansen11, Morten S. Olesen10, Morten S. Olesen11, Stefan Kääb12, Britt M. Beckmann12, Peter Weeke13, Hiroshi Watanabe14, Naoto Endo14, Tohru Minamino14, Minoru Horie15, Seiko Ohno15, Kanae Hasegawa15, Naomasa Makita16, Akihiko Nogami, Wataru Shimizu17, Takeshi Aiba, Philippe Froguel18, Philippe Froguel19, Philippe Froguel20, Beverley Balkau2, Beverley Balkau21, Olivier Lantieri22, Margherita Torchio5, Cornelia Wiese23, David Weber23, Rianne Wolswinkel1, Ruben Coronel1, Bas J. Boukens1, Stéphane Bézieau, Eric Charpentier3, Eric Charpentier2, Eric Charpentier4, Stéphanie Chatel, Aurore Despres, Françoise Gros2, Françoise Gros3, Françoise Gros4, Florence Kyndt, Simon Lecointe, Pierre Lindenbaum, Vincent Portero3, Vincent Portero4, Vincent Portero2, Jade Violleau, Manfred Gessler23, Hanno L. Tan1, Dan M. Roden13, Vincent M. Christoffels1, Hervé Le Marec, Arthur A.M. Wilde1, Vincent Probst, Jean-Jacques Schott, Christian Dina, Richard Redon 
TL;DR: The association signals at SCN5A-SCN10A demonstrate that genetic polymorphisms modulating cardiac conduction can also influence susceptibility to cardiac arrhythmia and indicate that common genetic variation can have a strong impact on the predisposition to rare diseases.
Abstract: Brugada syndrome is a rare cardiac arrhythmia disorder, causally related to SCN5A mutations in around 20% of cases. Through a genome-wide association study of 312 individuals with Brugada syndrome and 1,115 controls, we detected 2 significant association signals at the SCN10A locus (rs10428132) and near the HEY2 gene (rs9388451). Independent replication confirmed both signals (meta-analyses: rs10428132, P = 1.0 × 10(-68); rs9388451, P = 5.1 × 10(-17)) and identified one additional signal in SCN5A (at 3p21; rs11708996, P = 1.0 × 10(-14)). The cumulative effect of the three loci on disease susceptibility was unexpectedly large (Ptrend = 6.1 × 10(-81)). The association signals at SCN5A-SCN10A demonstrate that genetic polymorphisms modulating cardiac conduction can also influence susceptibility to cardiac arrhythmia. The implication of association with HEY2, supported by new evidence that Hey2 regulates cardiac electrical activity, shows that Brugada syndrome may originate from altered transcriptional programming during cardiac development. Altogether, our findings indicate that common genetic variation can have a strong impact on the predisposition to rare diseases.

467 citations

Journal ArticleDOI
01 Nov 2002
TL;DR: In this paper, the authors evaluated the Mendelian autosomal-dominant inheritance with an electrocardiogram (ECG) and echocardiogram and found that the risk of atrial fibrillation (AF) or left atrial enlargement carries a significant risk of thromboembolism.
Abstract: When an individual is diagnosed with hypertrophic cardiomyopathy (HCM), all relatives potentially affected by Mendelian autosomal-dominant inheritance should be evaluated with an electrocardiogram (ECG) and echocardiogram. Genetic testing should be considered in high-risk mutations where there are diagnostic uncertainties. Symptom relief depends on β-blockers as first-line therapy. If the disease is nonobstructive, then calcium channel blockers can be added or used alone. If there is a significant left ventricular outflow tract (LVOT) gradient then disopyramide can be used, ideally in combination with a β-blocker. Verapamil should be used with care due to potential exacerbation of the LVOT gradient. Nonmedical therapy for obstructive disease consists of surgical myectomy, alcohol septal ablation, or dual-chamber pacing. Surgery is the gold standard, although in experienced hands and directed appropriately, septal ablation achieves good results. Pacing is generally less effective. The development of atrial fibrillation (AF) or left atrial enlargement carries a significant risk of thromboembolism. All patients should be closely observed for AF and thromboembolic risk, and the threshold for initiation of anticoagulation should be low in patients with sustained palpitations, atrial enlargement, and nonsustained supraventricular arrhythmia on Holter. All patients with HCM should be assessed for their risk of sudden death regardless of severity of symptoms or morphology. The factors predictive of risk are 1) previous cardiac arrest; 2) unexplained syncope; 3) family history of premature sudden death; 4) abnormal blood pressure response to exercise; 5) nonsustained ventricular tachycardia; and 6) severe left ventricular hypertrophy ≥ 30 mm.

415 citations

Journal ArticleDOI
TL;DR: Over half of SADS deaths were likely to be due to inherited heart disease; accurate identification is vital for appropriate prophylaxis amongst relatives who should undergo comprehensive cardiological evaluation, guided and confirmed by mutation analysis.
Abstract: Aims At least 4% of sudden deaths are unexplained at autopsy [sudden arrhythmic death syndrome (SADS)] and a quarter may be due to inherited cardiac disease. We hypothesized that comprehensive clinical investigation of SADS families would identify more susceptible individuals and causes of death. Methods and results Fifty seven consecutively referred families with SADS death underwent evaluation including resting 12 lead, 24 h and exercise ECG and 2D echocardiography. Other investigations included signal averaged ECG, ajmaline testing, cardiac magnetic resonance imaging, and mutation analysis. First-degree relatives [184/262 (70%)] underwent evaluation, 13 (7%) reporting unexplained syncope. Seventeen (30%) families had a history of additional unexplained premature sudden death(s). Thirty families (53%) were diagnosed with inheritable heart disease: 13 definite long QT syndrome (LQTS), three possible/probable LQTS, five Brugada syndrome, five arrhythmogenic right ventricular cardiomyopathy (ARVC), and four other cardiomyopathies. One SCN5A and four KCNH2 mutations (38%) were identified in 13 definite LQTS families, one SCN5A mutation (20%) in five Brugada syndrome families and one (25%) PKP2 (plakophyllin2) mutation in four ARVC families. Conclusion Over half of SADS deaths were likely to be due to inherited heart disease; accurate identification is vital for appropriate prophylaxis amongst relatives who should undergo comprehensive cardiological evaluation, guided and confirmed by mutation analysis.

385 citations


Cited by
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Journal ArticleDOI
TL;DR: Authors/Task Force Members: Piotr Ponikowski* (Chairperson) (Poland), Adriaan A. Voors* (Co-Chair person) (The Netherlands), Stefan D. Anker (Germany), Héctor Bueno (Spain), John G. F. Cleland (UK), Andrew J. S. Coats (UK)

13,400 citations

Journal ArticleDOI
TL;DR: ACCF/AHAIAI: angiotensin-converting enzyme inhibitor as discussed by the authors, angio-catabolizing enzyme inhibitor inhibitor inhibitor (ACS inhibitor) is a drug that is used to prevent atrial fibrillation.
Abstract: ACC/AHA : American College of Cardiology/American Heart Association ACCF/AHA : American College of Cardiology Foundation/American Heart Association ACE : angiotensin-converting enzyme ACEI : angiotensin-converting enzyme inhibitor ACS : acute coronary syndrome AF : atrial fibrillation

7,489 citations

Journal ArticleDOI
TL;DR: WRITING GROUP MEMBERS Emelia J. Benjamin, MD, SCM, FAHA Michael J. Reeves, PhD Matthew Ritchey, PT, DPT, OCS, MPH Carlos J. Jiménez, ScD, SM Lori Chaffin Jordan,MD, PhD Suzanne E. Judd, PhD
Abstract: WRITING GROUP MEMBERS Emelia J. Benjamin, MD, SCM, FAHA Michael J. Blaha, MD, MPH Stephanie E. Chiuve, ScD Mary Cushman, MD, MSc, FAHA Sandeep R. Das, MD, MPH, FAHA Rajat Deo, MD, MTR Sarah D. de Ferranti, MD, MPH James Floyd, MD, MS Myriam Fornage, PhD, FAHA Cathleen Gillespie, MS Carmen R. Isasi, MD, PhD, FAHA Monik C. Jiménez, ScD, SM Lori Chaffin Jordan, MD, PhD Suzanne E. Judd, PhD Daniel Lackland, DrPH, FAHA Judith H. Lichtman, PhD, MPH, FAHA Lynda Lisabeth, PhD, MPH, FAHA Simin Liu, MD, ScD, FAHA Chris T. Longenecker, MD Rachel H. Mackey, PhD, MPH, FAHA Kunihiro Matsushita, MD, PhD, FAHA Dariush Mozaffarian, MD, DrPH, FAHA Michael E. Mussolino, PhD, FAHA Khurram Nasir, MD, MPH, FAHA Robert W. Neumar, MD, PhD, FAHA Latha Palaniappan, MD, MS, FAHA Dilip K. Pandey, MBBS, MS, PhD, FAHA Ravi R. Thiagarajan, MD, MPH Mathew J. Reeves, PhD Matthew Ritchey, PT, DPT, OCS, MPH Carlos J. Rodriguez, MD, MPH, FAHA Gregory A. Roth, MD, MPH Wayne D. Rosamond, PhD, FAHA Comilla Sasson, MD, PhD, FAHA Amytis Towfighi, MD Connie W. Tsao, MD, MPH Melanie B. Turner, MPH Salim S. Virani, MD, PhD, FAHA Jenifer H. Voeks, PhD Joshua Z. Willey, MD, MS John T. Wilkins, MD Jason HY. Wu, MSc, PhD, FAHA Heather M. Alger, PhD Sally S. Wong, PhD, RD, CDN, FAHA Paul Muntner, PhD, MHSc On behalf of the American Heart Association Statistics Committee and Stroke Statistics Subcommittee Heart Disease and Stroke Statistics—2017 Update

7,190 citations

Journal ArticleDOI
TL;DR: Authors/Task Force Members: Piotr Ponikowski* (Chairperson) (Poland), Adriaan A. Voors* (Co-Chair person) (The Netherlands), Stefan D. Anker (Germany), Héctor Bueno (Spain), John G. F. Cleland (UK), Andrew J. S. Coats (UK)
Abstract: ACC/AHA : American College of Cardiology/American Heart Association ACCF/AHA : American College of Cardiology Foundation/American Heart Association ACE : angiotensin-converting enzyme ACEI : angiotensin-converting enzyme inhibitor ACS : acute coronary syndrome AF : atrial fibrillation

6,757 citations

Journal ArticleDOI
TL;DR: Author(s): Writing Group Members; Mozaffarian, Dariush; Benjamin, Emelia J; Go, Alan S; Arnett, Donna K; Blaha, Michael J; Cushman, Mary; Das, Sandeep R; de Ferranti, Sarah; Despres, Jean-Pierre; Fullerton, Heather J; Howard, Virginia J; Huffman, Mark D; Isasi, Carmen R; Jimenez, Monik C; Judd, Suzanne
Abstract: Author(s): Writing Group Members; Mozaffarian, Dariush; Benjamin, Emelia J; Go, Alan S; Arnett, Donna K; Blaha, Michael J; Cushman, Mary; Das, Sandeep R; de Ferranti, Sarah; Despres, Jean-Pierre; Fullerton, Heather J; Howard, Virginia J; Huffman, Mark D; Isasi, Carmen R; Jimenez, Monik C; Judd, Suzanne E; Kissela, Brett M; Lichtman, Judith H; Lisabeth, Lynda D; Liu, Simin; Mackey, Rachel H; Magid, David J; McGuire, Darren K; Mohler, Emile R; Moy, Claudia S; Muntner, Paul; Mussolino, Michael E; Nasir, Khurram; Neumar, Robert W; Nichol, Graham; Palaniappan, Latha; Pandey, Dilip K; Reeves, Mathew J; Rodriguez, Carlos J; Rosamond, Wayne; Sorlie, Paul D; Stein, Joel; Towfighi, Amytis; Turan, Tanya N; Virani, Salim S; Woo, Daniel; Yeh, Robert W; Turner, Melanie B; American Heart Association Statistics Committee; Stroke Statistics Subcommittee

6,181 citations