Long-term fate of long QT syndrome patients diagnosed in childhood and differential effects of beta-blockers

Figures

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  Figure 1. Distribution of the presentation age.

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  Figure 2. Genotype distribution in relation to the presentation age newborns vs. older children, LQTS 2 and 3 more prevalent in those presenting as newborns (p = 0.042).

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  Figure 3. Survival probability according to genotype.

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  Figure 4. Freedom from MAE according to genotype.

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  Figure 5. Freedom from MAE according to presentation age.

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  Figure 6. Hazard ratio estimates for prediction of MAE: demographic and clinical factors.

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  Figure 7. Hazard ratio estimates for prediction of MAE: non-selective vs. selective beta-blockers.

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  Figure 8. Beta-blocker type and cumulative MAE burden according to time on therapy. Analysis performed using Poisson regression model. Crosses (x) indicate a period of treatment with selective, whereas dots (·) indicate non-selective BBs. Circles around symbols represent the number of MAEs experienced during these periods. The size of the circle is proportional to the number of episodes. Isochrones indicate the estimated number of MAEs at respective treatment durations. During the 10-year treatment period, patients receiving non-selective BBs experienced 0.085 MAE episodes per patient as compared to 0.327 in patients on selective BB (12); the p-value for the difference between BB types is p = 0.011 for a mode assuming independence and p = 0.007 for a mixed model considering patient random effect.

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  Figure 9. Cumulative MAE burden along with beta-blocker type, according to age at presentation and time on therapy. Analysis performed using a Poisson regression model. MAE episodes are most frequent in patients presenting early in life and occur, except few patients presenting as neonates, almost exclusively on selective beta-blockers. For a detailed explanation, see text and the legend for Figure 8.