Key result
Sick sinus syndrome occurred in 10.5% of children with hypertrophic cardiomyopathy and strongly predicted life-threatening arrhythmias (OR 5.18; p<0.001), which affected 13.5% overall.
Why the study?
Rhythm and conduction disorders are key determinants of prognosis in hypertrophic cardiomyopathy, motivating assessment of their nature, frequency, and clinical-prognostic significance in affected children.
Cohort (n=200)
In children with hypertrophic cardiomyopathy, rhythm and conduction disorders are highly prevalent (77%), and sick sinus syndrome confers a more than fivefold increased odds of life-threatening arrhythmias, supporting its inclusion in pediatric sudden cardiac death risk stratification.
Sick sinus syndrome was associated with life-threatening arrhythmias in pediatric hypertrophic cardiomyopathy; hypothesis-generating and requires prospective validation before guiding care.
Rhythm and conduction disorders remain key determinants of prognosis in hypertrophic cardiomyopathy. Objective. To assess the nature, frequency, and clinical-prognostic significance of cardiac rhythm and conduction disorders in children with hypertrophic cardiomyopathy. Materials and Methods . This retrospective-prospective study included 200 children with confirmed primary hypertrophic cardiomyopathy (median age 14.34 years; 2/3 male). Median follow-up was 5.74 years. Life-threatening arrhythmias were defined as sudden cardiac death, aborted sudden cardiac death, or appropriate implantable cardioverter-defibrillator therapy. Results. Rhythm and conduction disorders were detected in 77 % of children. Ventricular extrasystole ≥ 10/24h was observed in 25.5 %, and non-sustained ventricular tachycardia in 20.5 %. Life-threatening arrhythmias occurred in 27 children (13.5 %): sudden cardiac death (n = 6), aborted sudden cardiac death (n = 11), and appropriate implantable cardioverter-defibrillator shocks for primary prevention (n = 10). Ventricular extrasystole 10–500/24h (OR 4.93; p = 0.00002) and ventricular extrasystole ≥ 500/24h (OR 6.84; p = 0.015) were associated with non-sustained ventricular tachycardia. Extreme myocardial hypertrophy increased the odds of non-sustained ventricular tachycardia 2.3-fold (p = 0.018). Sick sinus syndrome was diagnosed in 10.5 % of children and was a strong predictor of life-threatening arrhythmias (OR 5.18; p<0.001). Intraventricular conduction disturbances were noted in 54.5 %, first-degree atrioventricular block in 20 %, and supraventricular tachyarrhythmias in 5.5 %. Conclusion . Hypertrophic cardiomyopathy is characterized by a high prevalence of rhythm and conduction disorders. Even moderate ventricular extrasystole is associated with non-sustained ventricular tachycardia, justifying routine Holter monitoring in all children with hypertrophic cardiomyopathy. Sick sinus syndrome is associated with a fivefold increased risk of life-threatening arrhythmias and should be incorporated into sudden cardiac death risk stratification algorithms. Such patients may warrant a lower 5-year sudden cardiac death risk threshold for dual-chamber cardioverter-defibrillator implantation.
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Presova et al. (2026) conducted a cohort in Primary hypertrophic cardiomyopathy in children (n=200). Sick sinus syndrome and rhythm/conduction disorders vs. Absence of sick sinus syndrome or specific rhythm/conduction disorders was evaluated on Life-threatening arrhythmias (sudden cardiac death, aborted sudden cardiac death, or appropriate implantable cardioverter-defibrillator therapy) (OR 5.18, p=<0.001). Sick sinus syndrome occurred in 10.5% of children with hypertrophic cardiomyopathy and strongly predicted life-threatening arrhythmias (OR 5.18; p<0.001), which affected 13.5% overall.
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