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Purpose of review The present article serves to review current risk assessment guidelines for sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) and to discuss how these guidelines can be applied to patients with childhood HCM. New diagnostic techniques that could lead to more accurate risk assessment tools are also discussed. Recent findings Current guidelines for risk assessment in childhood HCM are extrapolated from adult guidelines and lack background research to validate their use. Continuous variables, such as wall thickness, are converted to binary variables, which is particularly concerning in pediatric patients’ where weight gain and linear growth is likely to lead to more significant hemodynamic changes in shorter periods of time. Some studies have even shown that risk factors concerning in adults may actually be protective in pediatric patients. Additionally, large gaps still remain between genotype and phenotype expression in HCM. Summary A better understanding of the relationship between cause, phenotype, and outcomes is needed to truly be able to determine risk for SCD in childhood HCM. Larger studies, including newer technologies and quantitative models, similar to the European HCM Risk-SCD model, which allows for a quantitative risk diagnosis, are needed as well.

The unique design of the NobleStitch™ EL allows it to be used to close residual defects following failed device PFO closure without impacting the integrity of previously placed double‐disk Gore occluders.

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