Key result
Childhood hypertrophic cardiomyopathy literature overrepresents worst-case scenarios, lacking robust population-based outcome studies.
Why the study?
Few population studies have been published on childhood hypertrophic cardiomyopathy despite its variable clinical spectrum and reports focusing on severe cases.
The spectrum of hypertrophic cardiomyopathy (HCM) ranges from silent phenotypes, typically with mild hypertrophy in asymptomatic patients, to aggressive phenotypes with malignant arrhythmias or hemodynamic compromise in patients with severe hypertrophy and disabling symptoms. This is true for adult patients as well as for children with HCM. Reports depicting worst-case scenarios of severe progressive hypertrophy and clinical deterioration in childhood tend to dominate the literature on childhood HCM, and few population studies have been published.
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Munk et al. (2017) conducted a review in Hypertrophic cardiomyopathy. Family screening was evaluated. The literature on childhood hypertrophic cardiomyopathy is dominated by worst-case scenarios of severe progressive hypertrophy, with few population studies published.
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