Elijah R Behr

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Developed in partnership with the Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA), a registered branch of the European Society of Cardiology, and the Asia Pacific Heart Rhythm Society (APHRS); and in collaboration with the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the Pediatric and(More)
BACKGROUND Mutations in KCNJ2, the gene encoding the inward-rectifying K+ channel Kir2.1, cause the cardiac, skeletal muscle, and developmental phenotypes of Andersen-Tawil syndrome (ATS; also known as Andersen syndrome). Although pathogenic mechanisms have been proposed for select mutations, a common mechanism has not been identified. METHODS Seventeen(More)
BACKGROUND Drug-induced long-QT syndrome (diLQTS) is an adverse drug effect that has an important impact on drug use, development, and regulation. We tested the hypothesis that common variants in key genes controlling cardiac electric properties modify the risk of diLQTS. METHODS AND RESULTS In a case-control setting, we included 176 patients of European(More)
and Management of Patients with Inherited Primary Arrhythmia Syndromes Silvia G. Priori, MD, PhD, (HRS Chairperson), Arthur A. Wilde, MD, PhD, (EHRA Chairperson), Minoru Horie, MD, PhD, (APHRS Chairperson), Yongkeun Cho, MD, PhD, (APHRS Chairperson), Elijah R. Behr, MA, MBBS, MD, FRCP, Charles Berul, MD, FHRS, CCDS, Nico Blom, MD, PhD*, Josep Brugada, MD,(More)
http://heart.bmj.com/cgi/content/full/87/2/169 Updated information and services can be found at: These include: Data supplement http://heart.bmj.com/cgi/content/full/87/2/169/DC1 "Web only references" References http://heart.bmj.com/cgi/content/full/87/2/169#otherarticles 16 online articles that cite this article can be accessed at:(More)
4.1% of sudden cardiac deaths in the 16-64 age-group are unexplained. In this group, cardiac pathological findings are normal and toxicological tests are negative; termed sudden arrhythmic death syndrome (SADS). We searched for evidence of inherited cardiac disease in cases of SADS. Of 147 first-degree relatives of 32 people who died of SADS, 109 (74%)(More)
Height is a classic complex trait with common variants in a growing list of genes known to contribute to the phenotype. Using a genecentric genotyping array targeted toward cardiovascular-related loci, comprising 49,320 SNPs across approximately 2000 loci, we evaluated the association of common and uncommon SNPs with adult height in 114,223 individuals from(More)
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(More)
OBJECTIVES The phenotypic triad of arrhythmogenic right ventricular cardiomyopathy (ARVC) associated with palmoplantar keratoderma and woolly hair has been previously associated with homozygous mutations in both plakoglobin and desmoplakin, which are both critical components of the desmosome. We present here a clinical and genetic study of a consanguineous(More)