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Another piece of the puzzle: Assessment of mechanical dispersion by magnetic resonance in long QT syndrome

  • Elena Arbelo
    Correspondence
    Address reprint requests and correspondence: Dr Elena Arbelo, Hospital Clínic de Barcelona, C/ Villarroel 170, Planta 6–Escala 3, 08036, Barcelona, Spain.
    Affiliations
    Institut Clínic Cardiovascular (ICCV) Hospital Clínic, Universitat de Barcelona, Barcelona, Spain and Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
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      Congenital long QT syndrome (LQTS) has traditionally been considered a purely electrical disease caused by mutations in genes encoding cardiac ion channels, which lead to a prolongation and dispersion of the action potential duration (APD) that may trigger early afterdepolarizations and, as a consequence, malignant ventricular arrhythmias. However, this classical concept of LQTS as a primary electrical disease has been challenged since the early 1990s. The first observations, using M-mode echocardiography, showed an abnormal myocardial contraction LQTS patients with a rapid early contraction phase followed by a slow late movement,
      • Nador F.
      • Beria G.
      • De Ferrari G.M.
      • Stramba-Badiale M.
      • Locati E.H.
      • Lotto A.
      • Schwartz P.J.
      Unsuspected echocardiographic abnormality in the long QT syndrome. Diagnostic, prognostic, and pathogenetic implications.
      which was corrected by the use of intravenous verapamil.
      • De Ferrari G.M.
      • Nador F.
      • Beria G.
      • Sala S.
      • Lotto A.
      • Schwartz P.J.
      Effect of calcium channel block on the wall motion abnormality of the idiopathic long QT syndrome.
      Nakayama et al demonstrated a regionally inhomogeneous and prolonged contraction time, which was directly correlated with the QTc.
      • Nakayama K.
      • Yamanari H.
      • Otsuka F.
      • Fukushima K.
      • Saito H.
      • Fujimoto Y.
      • Emori T.
      • Matsubara H.
      • Uchida S.
      • Ohe T.
      Dispersion of regional wall motion abnormality in patients with long QT syndrome.
      Later on, tissue Doppler, strain, and speckle-tracking echocardiography showed that LQTS patients had a prolonged myocardial contraction duration and mechanical dispersion both longitudinally and transmurally.
      • Savoye C.
      • Klug D.
      • Denjoy I.
      • Ennezat P.V.
      • Le Tourneau T.
      • Guicheney P.
      • Kacet S.
      Tissue Doppler echocardiography in patients with long QT syndrome.
      • Haugaa K.H.
      • Edvardsen T.
      • Leren T.P.
      • Gran J.M.
      • Smiseth O.A.
      • Amlie J.P.
      Left ventricular mechanical dispersion by tissue Doppler imaging: a novel approach for identifying high-risk individuals with long QT syndrome.
      • Haugaa K.H.
      • Amlie J.P.
      • Berge K.E.
      • Leren T.P.
      • Smiseth O.A.
      • Edvardsen T.
      Transmural differences in myocardial contraction in long-QT syndrome. mechanical consequences of ion channel dysfunction.
      • Leren I.S.
      • Hasselberg N.E.
      • Saberniak J.
      • Håland T.F.
      • Kongsgård E.
      • Smiseth O.A.
      • Edvardsen T.
      • Haugaa K.H.
      Cardiac mechanical alterations and genotype specific differences in subjects with long QT syndrome.
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