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Vol. 73. Issue 1.
Pages 88 (January 2020)
ECG Contest
DOI: 10.1016/j.rec.2019.05.012
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Response to ECG, December 2019
Respuesta al ECG de diciembre de 2019
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Francisco Ribesa,
Corresponding author
fribestur@gmail.com

Corresponding author:
, Antonio Marcoa, Antonio Sánchezb
a Servicio de Cardiología, Hospital Universitario de Sant Joan, Sant Joan d’Alacant, Alicante, Spain
b Sección de Cardiología Pediátrica, Servicio de Pediatría, Hospital Universitari i Politècnic La Fe, Valencia, Spain
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Rev Esp Cardiol. 2019;72:107910.1016/j.rec.2019.05.013
Francisco Ribes, Antonio Marco, Antonio Sánchez
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This patient has a marked QT prolongation (≈ 520 ms) with T-wave macroalternans, and he presented with a cardiorespiratory arrest from which he receoved,1 and so answer 1 is incorrect. This is an autosomal-recessive condition with a poor prognosis, which is more frequent when consanguinity is present, and which characteristically includes congenital deafness2 (answer 2, correct). Given the family history of sudden cardiac death in early childhood, and the electrocardiographic abnormalities observed, diagnosis of obstructive hypertrophic cardiomyopathy is unlikely (answer 4, incorrect). Timothy syndrome, although a congenital long QT syndrome, is an autosomal-dominant form that is associated with other disorders, such as mental retardation, autism, dysmorphy, calcium metabolism disorders, and immunodeficiency, and so answer 3 is also incorrect.

References
[1]
I. Goldenberg, A.J. Moss, D.R. Peterson, et al.
Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome.
Circulation., 117 (2008), pp. 2184-2191
[2]
J.R. Giudicessi, M.J. Ackerman.
Prevalence and potential genetic determinants of sensorineural deafness in KCNQ1 homozygosity and compound heterozygosity.
Circ Cardiovasc Genet., 6 (2013), pp. 193-200
Copyright © 2019. Sociedad Española de Cardiología
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