Progressive Left Ventricular Remodeling for Predicting Mortality in Children With Dilated Cardiomyopathy: The Pediatric Cardiomyopathy Registry

Figures

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  Figure 1. Cohort study flow chart. Cohort selection of children with dilated cardiomyopathy from the Pediatric Cardiomyopathy Registry in a study to determine the prognostic value of serial changes in echocardiographic measurements. *Children not having an echocardiogram within 90 days before death or cardiac transplant in the first year, or within 6 months of their 1‐year anniversary of the first echocardiogram, if event free during the first year.

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  Figure 2. Progression of LV dimension Z score. Left ventricular end‐diastolic dimension Z scores at (A) diagnosis, (B) 1 year later, and (C) changes between baseline and 1 year for children with dilated cardiomyopathy who died, underwent heart transplant, or survived during the first year after diagnosis. Data in the box‐and‐whisker plots are medians, interquartile ranges, minimum and maximum within median±1.5 interquartile range and outliers. Survivors differed significantly from both other groups at time points A and B. The changes in left ventricular end‐diastolic dimension Z scores between survivors, those transplanted, and nonsurvivors (C) were also significant. EDD indicates end‐diastolic dimension; and LV, left ventricular.

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  Figure 3. Progression of LV posterior wall thickness Z score. LVPWT Z scores at (A) diagnosis, (B) 1 year later, and (C) changes between diagnosis and 1 year for children with dilated cardiomyopathy who died, underwent heart transplant, or survived during the first year after diagnosis. Data in the box‐and‐whisker plots are medians, interquartile ranges, minimum and maximum within median±1.5 interquartile range and outliers. Differences were only marginally significant at baseline but were more evident at 1 year. Changes in left ventricular wall thickness (C) were similar in all 3 groups, with no clear between‐group differences from baseline. LVPWT indicates left ventricular end‐diastolic posterior wall thickness.

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  Figure 4. Progression of LV wall thickness to dimension ratio Z score. The ratio of left ventricular posterior wall thickness:left ventricular end diastolic dimension Z scores at (A) diagnosis, (B) 1 year later, and (C) changes between baseline and 1 year for children with dilated cardiomyopathy who died, underwent heart transplant, or survived during the first year after diagnosis. Data in the box‐and‐whisker plots are medians, interquartile ranges, minimum and maximum within median±1.5 interquartile range and outliers. Differences between the 3 groups were significant at both time points but not with regard to the magnitude of change during the first year shown in panel (C). LV indicates left ventricular.

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  Figure 5. Progression of LV fractional shortening Z score. Left ventricular fractional shortening Z scores at (A) diagnosis/(baseline), (B) 1 year later, and (C) changes between baseline and 1 year for children with dilated cardiomyopathy who died, underwent heart transplant, or survived during the first year after diagnosis. Data in the box‐and‐whisker plots are medians, interquartile ranges, minimum and maximum within median±1.5 interquartile range and outliers. The groups differed significantly at both time points, as well as in respect of the improvement in LVFS from 0 to 1 year (C). LVFS indicates left ventricular fractional shortening.

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  Figure 6. Transplant‐free survival probability distribution in children with dilated cardiomyopathy. A, From diagnosis to 12 months after baseline echocardiogram. B, For those surviving beyond 1 year after diagnosis (x axis is in years). Dotted lines are 95% confidence limits.