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Review
. 2012 Aug 1;5(4):868-77.
doi: 10.1161/CIRCEP.111.962019.

Long-QT syndrome: from genetics to management

Peter J Schwartz  1 , Lia Crotti, Roberto Insolia
Affiliations
Review

Long-QT syndrome: from genetics to management

Peter J Schwartz et al. Circ Arrhythm Electrophysiol. .

Erratum in

  • Circ Arrhythm Electrophysiol. 2012 Dec;5(6):e119-20
No abstract available

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Figures

Figure 1
Figure 1
Examples of T wave alternans from a 2-year old LQTS patient with multiple episodes of cardiac arrest. Tracings are from a 24-h Holter recording.
Figure 2
Figure 2
Kaplan-Meier curves of event-free survival comparing JLN patients vs LQT1, LQT2 and LQT3 symptomatic patients. (From ref. 5)
Figure 3
Figure 3
Triggers for all lethal and non-lethal cardiac events in the three genotypes. (Modified from ref. 41)
Figure 4
Figure 4
Unadjusted Kaplan-Meier estimate of the cumulative event-free survival (any first event) in the whole (non-SA– SA) A341V population plotted vs the LQT1 non-A341V group. Any cardiac event, whichever occurred first, was considered from birth through 40 years of age and before β-blocker therapy. Numbers at risk are indicated. (From ref. 13)
Figure 5
Figure 5
Cumulative event-free survival for a first appropriate ICD shock according to an increasing risk score (M-FACT) in (A) all patients and (B) in patients with no prior ACA. (From ref. 72)
See this image and copyright information in PMC

References

    1. Schwartz PJ, Periti M, Malliani A. The long Q-T syndrome. Am Heart J. 1975;89:378–390. - PubMed
    1. Romano C, Gemme G, Pongiglione R. Aritmie cardiache rare dell'età pediatrica. Clin Pediat. 1963;45:656–683. - PubMed
    1. Ward OC. A new familial cardiac syndrome in children. J Irish Med Ass. 1964;54:103–106. - PubMed
    1. Jervell A, Lange-Nielsen F. Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death. Am Heart J. 1957;54:59–68. - PubMed
    1. Schwartz PJ, Spazzolini C, Crotti L, Bathen J, Amlie JP, Timothy K, Shkolnikova M, Berul CI, Bitner-Glindzicz M, Toivonen L, Horie M, Schulze-Bahr E, Denjoy I. The Jervell and Lange-Nielsen Syndrome. Natural history, molecular basis, and clinical outcome. Circulation. 2006;113:783–790. - PubMed

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