Sudden cardiac death in a child with low-risk hypertrophic cardiomyopathy: limitations of current pediatric risk stratification—a case report
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ID: 319847
2026
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Abstract
Abstract Background Hypertrophic cardiomyopathy (HCM) is characterized by marked phenotypic heterogeneity and remains a leading cause of sudden cardiac death (SCD) in young individuals. Risk stratification in children is particularly challenging, and current predictive models may underestimate arrhythmic risk in certain patients. Case summary We report a familial case of sarcomeric HCM. A 41-year-old man presented with sustained ventricular tachycardia revealing asymmetric HCM, requiring implantable cardioverter–defibrillator (ICD) implantation, and later died from end-stage heart failure. His 12-year-old son was diagnosed with asymmetric HCM and was classified as low risk according to the HCM Risk-Kids score. At the time of evaluation, the child did not meet current guideline based criteria for ICD implantation. Nevertheless, SCD occurred during moderate physical exertion 2 months after diagnosis. Discussion This familial case highlights the limitations of paediatric risk stratification scores at the individual level and underscores the need for a more individualized approach integrating family history, early phenotypic expression, advanced imaging findings, and genetic context beyond risk scores alone.
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| Authors | Salma Bensalah, Amina Outahayou, Abdellah Sbai Sassi, Ibtissam Fellat, Mohamed Cherti |
| Journal | European Heart Journal - Case Reports |
| Year | 2026 |
| DOI |
10.1093/ehjcr/ytag458
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| URL | |
| Keywords | Keywords not found |
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