Elsevier

American Heart Journal

Volume 89, Issue 3, March 1975, Pages 378-390
American Heart Journal

The long Q-T syndrome

https://doi.org/10.1016/0002-8703(75)90089-7Get rights and content

Abstract

Recent clinical and experimental data on the long Q-T syndrome (LQTS) are presented and discussed. The pathogenesis of LQTS is dependent on an imbalance between various components of the cardiac sympathetic innervation. A congenital decreased activity through the right cardiac sympathetic nerves seems to be the more likely pathogenetic mechanism for the majority of cases. Other forms of sympathetic imbalance, including left or even right hyperactivity, are, however, possible in isolated cases.

Beta-blockers, at full blocking dose, represent the therapy of choice and are greatly effective in reducing the mortality (from 73 per cent to 6 per cent). If syncopal attacks are not eliminated by the medical therapy, then the ablation of the left stellate ganglion along with the first thoracic ganglia is the most rational and specific therapy. The possibility for the correctly diagnosed and treated patients to escape an otherwise impending death calls urgently for diffusion of the knowledge about the long Q-T syndrome.

References (105)

  • A. Langslet et al.

    Changes in ECG and cardiac rhythm with epilepsy-like attack and sudden death in childhood

    T. Norske Laegeforen

    (1974)
  • J.E. Fay et al.

    Surdo-cardiac syndrome: incidence among children in school for deaf

    Canad. Med. Assoc. J.

    (1971)
  • A. Wennevold et al.

    Adams-Stokes syndrome in children without organic heart disease

    Acta Med. Scandin.

    (1965)
  • C. Romano et al.

    Aritmie cardiache rare dell'età pediatrica

    Clin. Pediatr.

    (1963)
  • O.C. Ward

    New familial cardiac syndrome in children

    J. Ir. Med. Assoc.

    (1964)
  • P.J. Schwartz et al.

    Alternanza elettrica dell'onda T: Evidenza clinica e sperimentale dei suoi rapporti con il sistema nervoso simpatico. (Abstract)

  • P.J. Schwartz et al.

    Electrical alternation of the T-wave: clinical and experimental evidence of its relationship with the sympathetic nervous system and with the long Q-T syndrome

    Am. Heart J.

    (1974)
  • F. Yanowitz et al.

    Functional distribution of right and left stellate innervation to the ventricles: production of neurogenic electrocardiographic changes by unilateral alteration of sympathetic tone

    Circ. Res.

    (1966)
  • A.J. Moss et al.

    Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long Q-T interval syndrome

    N. Engl. J. Med.

    (1971)
  • F. Dessertenne et al.

    Un chapitre nouveau d'ECG: Les variations progressives de l'amplitude de l'electrocardiogramme

    Actual. Cardiol. Angeiol. Int.

    (1966)
  • W.E. Nance et al.

    The Jervell and Lange-Nielsen syndrome

  • G.R. Fraser et al.

    Congenital deafness associated with electrocardiographic abnormalities, fainting attacks and sudden death

    Q. J. Med.

    (1964)
  • G.R. Fraser et al.

    Genetical aspects of the cardio-auditory syndrome of Jervell and Lange-Nielsen (congenital deafness and electrocardiographic abnormalities)

    Ann. Hum. Genet.

    (1964)
  • A. Jervell

    Surdocardiac and related syndromes in children

    Adv. Intern. Med.

    (1971)
  • P. Froggatt et al.

    Sudden unexpected death in infants. Evidence on a lethal cardiac arrhythmia

    Ulster Med. J.

    (1973)
  • Jervell, A.: Personal communication,...
  • Å. Gyllensward

    Fits of unusual origin

    Acta Paediatr.

    (1957)
  • T. Moller

    Fits of unusual origin

    Acta Paediatr.

    (1957)
  • S.A. Levine et al.

    Congenital deafmutism, prolonged Q-T interval, syncopal attacks, and sudden death

    N. Engl. J. Med.

    (1958)
  • Romano, C.: Personal communication,...
  • Ward, O. C.: Personal communication,...
  • Froggatt, P.: Personal communication,...
  • S.A. Lisker et al.

    The cardio-auditory syndrome of Jervell and Lange-Nielsen: Report of an additional case with radioelectrocardiographic monitoring during exercise

    Am. J. Med. Sci.

    (1966)
  • A. Jervell et al.

    Surdo-cardialt syndrome

    Nord. Med.

    (1967)
  • M. Lamy et al.

    Le syndrome je Jervell et Lange-Nielsen (a propos de deux observations)

    Arch. Franc. Pediatr.

    (1967)
  • Frezal, J. and Vacheron, A.: Personal communication,...
  • Bonham-Carter, R. E.: Personal communication to P. Froggatt,...
  • H.C. Kallfelz

    Über ein neues EKG-Syndrom bei Kindern mit synkopalen Anfallen und plotzlichem Tod

    Dsch. Med. Wschr.

    (1968)
  • E. Tamura et al.

    J. Pediatr. Pract.

    (1969)
  • C. Dupuis et al.

    Le syndrome de Jervell et Lange-Nielsen. A propos d'une observation

    Arch. Mal. Coeur

    (1969)
  • C.L. Fauchier et al.

    Sur un cas de dyndrome de Jervell et Lange-Nielsen

    Pediatrie

    (1969)
  • A. Sanchez Cascos et al.

    Cardio-auditory syndromes. Cardiac and genetic study of 511 deaf-mute children

    Br. Heart J.

    (1969)
  • Sanchez-Cascos, A. Personal communication,...
  • G.E. Gale et al.

    Hereditary prolongation of Q-T interval. Study of two families

    Br. Heart J.

    (1970)
  • Barlow, J. B. Personal communication,...
  • Combrink, J. M. Personal communication to G. E. Gale,...
  • Karhunen, P. Personal communication,...
  • H. Lipp et al.

    Recurrent ventricular tachyarrhythmias in a patient with a prolonged Q-T interval

    Med. J. Aust.

    (1970)
  • Lipp, H. Personal communication,...
  • Phillips, J. Personal communication,...
  • Cited by (764)

    • Precision therapy in congenital long QT syndrome

      2024, Trends in Cardiovascular Medicine
    • Phenotypes of Overdiagnosed Long QT Syndrome

      2023, Journal of the American College of Cardiology
    • Genetic testing in athletes

      2023, Athlete's Heart: A Multimodal Approach - From Physiological to Pathological Cardiac Adaptations
    • Congenital Long QT Syndrome

      2022, JACC: Clinical Electrophysiology
    View all citing articles on Scopus
    View full text