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Case Reports
. 2020 Jan 25;6(5):256-260.
doi: 10.1016/j.hrcr.2020.01.007. eCollection 2020 May.

Electrical storm treated successfully in a patient with TANGO2 gene mutation and long QT syndrome: A case report

Frederico Scuotto  1 , Maria Fernanda Silva Jardim  1 , Flávia Balbo Piazzon  1 , Caio Marcos de Moraes Albertini  1 , Renato Samy Assad  1 , Guilherme Fenelon  1 , Claudio Cirenza  1
Affiliations
Case Reports

Electrical storm treated successfully in a patient with TANGO2 gene mutation and long QT syndrome: A case report

Frederico Scuotto et al. HeartRhythm Case Rep. .
No abstract available

Keywords: Cardioverter-defibrillator; Electrical storm; Implantable; Isoproterenol; Long QT syndrome; Pediatrics; Torsades de pointes.

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Figures

Figure 1
Figure 1
Electrocardiogram on the seventh day of hospitalization showing sinus rhythm and prolonged QT interval (520 ms).
Figure 2
Figure 2
Electrocardiogram on 10th day of hospitalization, showing the presence of pauses (red arrows) after the paired premature ventricular contractions, but not long enough to trigger a torsades de pointes episode.
Figure 3
Figure 3
Holter tracing on the 11th day of hospitalization, showing that the torsades de pointes episode that degenerates into ventricular fibrillation was not pause-dependent.
See this image and copyright information in PMC

References

    1. Schwartz P.J., Stramba-Badiale M., Crotti L. Prevalence of the congenital long-QT syndrome. Circulation. 2009;120:1761–1767. - PMC - PubMed
    1. Zipes D.P., Jalife J. Elsevier; Philadelphia: 2009. Cardiac Electrophysiology: From Cell to the Bedside. 5th ed; pp. 731–743.
    1. Kremer L.S., Distelmaier F., Alhaddad B. Bi-allelic truncating mutations in TANGO2 cause infancy-onset recurrent metabolic crises with encephalocardiomyopathy. Am J Hum Genet. 2016;98:358–362. - PMC - PubMed
    1. Lalani S.R., Liu P., Rosenfeld J.A. Recurrent muscle weakness with rhabdomyolysis, metabolic crises, and cardiac arrhythmia due to bi-allelic TANGO2 mutations. Am J Hum Genet. 2016;98:347–357. - PMC - PubMed
    1. Clausen H., Pflaumer A., Kamberi S., Davis A. Electrical storm in children. Pacing Clin Electrophysiol. 2013;36:391–401. - PubMed

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