Unveiling Clinical and Genetic Distinctions in Pure‐Apical Versus Distal‐Dominant Apical Hypertrophic Cardiomyopathy

Figures

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  Figure 1. Cumulative incidence for HCM‐related and non–HCM‐related deaths. This figure illustrates the cumulative incidence of HCM‐related deaths and non–HCM‐related deaths in patients with PA and DD subtypes of HCM. Solid lines represent HCM‐related deaths, while dashed lines represent non–HCM‐related deaths. Blue lines indicate the PA subtype, and red lines indicate the DD subtype. The P values for Gray's K‐sample test are displayed in the figure. The table below the graph provides the cumulative incidence rates at 5, 10, 15, 20, and 25 y for each group and event type. Additionally, Gray's K‐sample test statistics and P values are summarized in the lower table. DD indicates distal‐dominant; HCM, hypertrophic cardiomyopathy; and PA, pure‐apical.

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  Figure 2. Comparison of incidence of HCM‐related events between the PA and DD groups. Kaplan–Meier curves for the incidence of HCM‐related events. A, Composite events, which included HCM‐related deaths, SCD relevant events, heart failure events, and embolic events. B, SCD relevant events, including SCD, successful resuscitation from VT or VF, and appropriate ICD discharge. C, Heart failure events including heart failure death and heart failure hospitalization. D, Embolic events, including embolic stroke and systemic embolism. DD indicates distal‐dominant; HCM, hypertrophic cardiomyopathy; HF, heart failure; PA, pure‐apical; SCD, sudden cardiac death; VF, ventricular fibrillation; and VT, ventricular tachycardia.

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  Figure 3. Time‐dependent morphological changes, left ventricular remodeling, and incidence of adverse events. This figure illustrates the morphological changes and LV remodeling observed in the PA and DD groups over the course of the follow‐up period. It also shows the incidence of adverse events in each group. The numbers in parentheses inside the colored squares indicate the sample sizes (N). DD indicates distal‐dominant; HF, heart failure; PA, pure‐apical; and SCD, sudden cardiac death.

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  Figure 4. Variant detection rates in the pure‐apical and distal‐dominant groups. This figure shows the detection rates of likely pathogenic and pathogenic variants in the pure‐apical and distal‐dominant groups, the rate being significantly higher in the latter group. The detection rates of variants of uncertain significance, likely pathogenic and pathogenic variants within genes associated with HCM were also higher in the distal‐dominant group. HCM indicates hypertrophic cardiomyopathy; and VUS, variant of uncertain significance.

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  Figure 5. Incidences of HCM‐related deaths and events in the variant‐positive and variant‐negative groups. This figure shows Kaplan–Meier curves depicting the incidence of HCM‐related deaths and events in 72 patients with ApHCM who underwent genetic testing. A, Occurrence of HCM‐related deaths. B, Composite events including HCM‐related deaths, events relevant to SCD, HF events, and embolic events. ApHCM indicates apical hypertrophic cardiomyopathy; HCM, hypertrophic cardiomyopathy; HF, heart failure; and SCD, sudden cardiac death.