Is long QT syndrome a disease of abnormal mechanical contraction?

نویسنده

  • David S Rosenbaum
چکیده

It is generally assumed that long QT syndrome (LQTS) is an arrhythmic disorder that occurs in patients with normal left ventricular systolic function. The present article by Haugaa et al challenges this assumption.1 One hundred and one patients with genetically documented LQTS were systematically compared to an age-matched unaffected control population for differences in regional contraction patterns as assessed by the speckled tracking method. Among the LQTS population, 64 had LQT1, 26 had LQT2, 1 had LQT3, 1 had LQT5, and 9 had documented Jervell and Lange Nielsen Syndrome. About half of all patients with LQTS were symptomatic. Various metrics of regional left ventricular contraction and spatial inhomogeneity of contraction were assessed. In addition to global strain, mean contraction duration measured separately in the longitudinal and circumferential directions was assessed as a mechanical counterpart to action potential duration or QT interval, dispersion of longitudinal or circumferential contraction duration served as a mechanical counterpart to regional (eg, apex versus base) dispersion of repolarization, and differences between longitudinal and circumferential contraction duration within individual regions served as a mechanical counterpart to transmural dispersion of repolarization. The latter measure was based on the assumption that longitudinal and circumferential contraction arise selectively from endocardial and midmyocardial locations, respectively, across the transmural wall. This assumption, in turn, is based on the finding that subendocardium mainly consists of longitudinal fibers, whereas midmyocardium mainly consists of circumferentially oriented fibers.2

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عنوان ژورنال:
  • Circulation

دوره 122 14  شماره 

صفحات  -

تاریخ انتشار 2010