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Case Reports
. 2009 Dec;6(12):1814-7.
doi: 10.1016/j.hrthm.2009.08.032. Epub 2009 Aug 29.

Resuscitated sudden cardiac death in Andersen-Tawil syndrome

Kelly J Airey  1 , Susan P Etheridge, Rabi Tawil, Martin Tristani-Firouzi
Affiliations
Case Reports

Resuscitated sudden cardiac death in Andersen-Tawil syndrome

Kelly J Airey et al. Heart Rhythm. 2009 Dec.
No abstract available

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Figures

Figure 1
Figure 1
12 lead ECG rhythms strip obtained upon first evaluation in Pediatric Cardiology Clinic. Manual QTc 430 ms. Note prominent U waves in leads V2 and V3.
Figure 2
Figure 2
Holter monitor recording demonstrating bigeminy with nonsustained run of bidirectional VT (best seen in lead 3).
Figure 3
Figure 3
Loop recorder rhythm strip demonstrating the onset of rapid polymorphic VT, alternating with monomorphic VT, deteriorating into asystole.
See this image and copyright information in PMC

References

    1. Andersen ED, Krasilnikoff PA, Overvad H. Intermittent muscular weakness, extrasystoles, and multiple developmental anomalies. A new syndrome? Acta Paediatr Scand. 1971;60:559–564. - PubMed
    1. Sansone V, Griggs RC, Meola G, et al. Andersen's syndrome: A distinct periodic paralysis. Ann Neurol. 1997;42:305–312. - PubMed
    1. Tawil R, Ptacek LJ, Pavlakis SG, et al. Andersen's syndrome: Potassium-sensitive periodic paralysis, ventricular ectopy, and dysmorphic features. Ann Neurol. 1994;35:326–330. - PubMed
    1. Plaster NM, Tawil R, Tristani-Firouzi M, et al. Mutations in kir2.1 cause the developmental and episodic electrical phenotypes of Andersen's syndrome. Cell. 2001;105:511–519. - PubMed
    1. Chun TU, Epstein MR, Dick M, 2nd, et al. Polymorphic ventricular tachycardia and kcnj2 mutations. Heart Rhythm. 2004;1:235–241. - PubMed

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