Concealed long QT syndrome

Concealed long QT syndrome


The term concealed long QT syndrome, is used to indicate individuals with genotype of long QT syndrome and a phenotype with normal QT interval (corrected QT interval of 440 milliseconds or less). They are usually detected on family screening of those with manifest long QT syndrome. Goldenberg I et al [1] noted that of about three thousand four hundred genotyped subjects form seven multinational LQTS (long QT syndrome) registries, around four hundred and seventy had concealed long QT syndrome and around thousand four hundred had prolonged QT intervals. In this report, over thousand five hundred were unaffected family members.

The risk of sudden cardiac death or aborted cardiac arrest was ten times higher in those with concealed long QT syndrome than the unaffected family members (four percent vs four tenths of a percent). This is not withstanding the finding that those with manifest long QT syndrome had a much higher risk of sudden cardiac death or aborted cardiac arrest at fifteen percent. In concealed LQT syndrome, the risk of sudden cardiac death or aborted cardiac arrest was higher in those in LQTS1 and LQTS3 genotypes than in LQTS2 genotype. But unlike in manifest long QT syndrome, females were not shown to be at higher risk in concealed LQTS.

Reference

  1. Goldenberg I, Horr S, Moss AJ, Lopes CM, Barsheshet A, McNitt S, Zareba W, Andrews ML, Robinson JL, Locati EH, Ackerman MJ, Benhorin J, Kaufman ES, Napolitano C, Platonov PG, Priori SG, Qi M, Schwartz PJ, Shimizu W, Towbin JA, Vincent GM, Wilde AA, Zhang L. Risk for life-threatening cardiac events in patients with genotype-confirmed long-QT syndrome and normal-range corrected QT intervals. J Am Coll Cardiol. 2011;57:51-9.

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