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      Electrophysiologic substrate in congenital Long QT syndrome: noninvasive mapping with electrocardiographic imaging (ECGI).

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          Abstract

          Congenital Long QT syndrome (LQTS) is an arrhythmogenic disorder that causes syncope and sudden death. Although its genetic basis has become well-understood, the mechanisms whereby mutations translate to arrhythmia susceptibility in the in situ human heart have not been fully defined. We used noninvasive ECG imaging to map the cardiac electrophysiological substrate and examine whether LQTS patients display regional heterogeneities in repolarization, a substrate that promotes arrhythmogenesis.

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          Author and article information

          Journal
          Circulation
          Circulation
          1524-4539
          0009-7322
          Nov 25 2014
          : 130
          : 22
          Affiliations
          [1 ] From the Cardiac Bioelectricity and Arrhythmia Center (R.V., J.N.A.S., G.F.V.H., Y.R.) and Department of Biomedical Engineering (R.V., Y.R.), Washington University in St. Louis, Saint Louis, MO; Division of Pediatric Cardiology, Washington University School of Medicine/St. Louis Children's Hospital, St. Louis, MO (J.N.A.S., G.F.V.H., Y.R.); Cardiovascular Diseases, Mount Sinai St. Luke's Roosevelt, Icahn School of Medicine at Mount Sinai, New York, NY (K.A.D.); Department of Medicine and Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, TN (R.L.A., D.M.R.); CardioInsight Technologies, Cleveland, OH (M.S.); Bordeaux University Hospital, LIRYC institute, Pessac, France (F.S., M.H.); and the Department of Medicine (Cardiology), Washington University School of Medicine/Barnes-Jewish Hospital, St. Louis, MO (Y.R.).
          [2 ] From the Cardiac Bioelectricity and Arrhythmia Center (R.V., J.N.A.S., G.F.V.H., Y.R.) and Department of Biomedical Engineering (R.V., Y.R.), Washington University in St. Louis, Saint Louis, MO; Division of Pediatric Cardiology, Washington University School of Medicine/St. Louis Children's Hospital, St. Louis, MO (J.N.A.S., G.F.V.H., Y.R.); Cardiovascular Diseases, Mount Sinai St. Luke's Roosevelt, Icahn School of Medicine at Mount Sinai, New York, NY (K.A.D.); Department of Medicine and Department of Pharmacology, Vanderbilt University School of Medicine, Nashville, TN (R.L.A., D.M.R.); CardioInsight Technologies, Cleveland, OH (M.S.); Bordeaux University Hospital, LIRYC institute, Pessac, France (F.S., M.H.); and the Department of Medicine (Cardiology), Washington University School of Medicine/Barnes-Jewish Hospital, St. Louis, MO (Y.R.). rudy@wustl.edu.
          Article
          CIRCULATIONAHA.114.011359 NIHMS630720
          10.1161/CIRCULATIONAHA.114.011359
          25294783
          76860f35-33bf-46f7-befd-c423d6dadf74
          © 2014 American Heart Association, Inc.
          History

          ECG imaging,arrhythmia,electrophysiology,imaging, diagnostic,long-QT syndrome

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