The Outcome of Long QT Syndrome, a Korean Single Center Study

Figures

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  Figure 1. The mode of initial presentation in the symptomatic group.

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  Figure 2. The distributions of genetic mutations are confirmed in the genetically verified group. G−/P+ = genotype negative and phenotype positive; JLNS = Jervell Lange-Nielsen syndrome; LQT1 = congenital long QT syndrome type 1; LQT2 = congenital long QT syndrome type 2; LQT3 = congenital long QT syndrome type 3; LQT4 = congenital long QT syndrome type 4; LQT7 = congenital long QT syndrome type 7; LQT8 = congenital long QT syndrome type 8; LQTM = multiple long QT syndrome-associated mutations; VUS = variant of uncertain significance.

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  Figure 3. BCE-free survival curve for entire genetically verified LQTS. (A) BCE-free survival through each genotype, statistical significance was analyzed except for LQT4, 7, 8 which were rare. (B) When comparing LQT1, 2, and 3, which account for most of LQTS, the prognosis was poor in the order of LQT3, LQT2, and LQT1. BCE = breakthrough cardiac event; G−/P+ = genotype negative and phenotype positive; LQTS = long QT syndrome; LQT1 = congenital long QT syndrome type 1; LQT2 = congenital long QT syndrome type 2; LQT3 = congenital long QT syndrome type 3; LQT4 = congenital long QT syndrome type 4; LQT7 = congenital long QT syndrome type 7; LQT8 = congenital long QT syndrome type 8; LQTM = multiple LQTS-associated mutations; VUS = variant of uncertain significance.

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  Figure 4. Individual/cumulative risk factors for appropriate ICD shock. Among the ICD recipients, the Kaplan-Meier curves were analyzed through the risk factors, primary vs. secondary prevention (A), QTc ≥500 ms (B), and family history of sudden cardiac death (C) for the event that received appropriate shock treatment. ICD = implantable cardioverter-defibrillator; QTc = corrected QT interval.