Abstract
[first paragraph of article]The true prevalence of hypertrophic cardiomyopathy (HCM) in childhood is unknown, but population-based studies have reported an annual incidence between 0.24–0.47 per 100,000 children. The aetiology of disease is more heterogeneous than that seen in adult populations, with up to 30% of patients having an inborn error of metabolism, malformation syndrome or neuromuscular syndrome. However, as in adults, most cases are caused by mutations in the cardiac sarcomere protein genes, even in young children. The long-term outcome of childhood HCM is highly variable and has been shown to depend partly on the age of presentation and underlying aetiology. Outside of infancy, the most frequent cause of mortality is sudden cardiac death (SCD), and one of the greatest challenges in managing young patients with HCM is identifying those at greatest risk of an arrhythmic event.
Highlights
The true prevalence of hypertrophic cardiomyopathy (HCM) in childhood is unknown, but population-based studies have reported an annual incidence between 0.24–0.47 per 100,000 children[1,2,3]
No studies have reported a significant association between sudden cardiac death (SCD) and gender Presentation in infancy is associated with an increased risk of mortality secondary to congestive cardiac failure[5,8]
Outside infancy the majority of studies found no association with age and risk of SCD
Summary
The true prevalence of hypertrophic cardiomyopathy (HCM) in childhood is unknown, but population-based studies have reported an annual incidence between 0.24–0.47 per 100,000 children[1,2,3]. RISK FACTORS FOR SCD IN CHILDHOOD HCM Risk factors for SCD in adult HCM include prior ventricular fibrillation (VF) or sustained ventricular tachycardia (VT); family history of sudden cardiac death; unexplained syncope; non-sustained ventricular tachycardia (NSVT); maximal left ventricular wall thickness >30 mm; left atrial dilatation; and left ventricular outflow tract obstruction. These conventional clinical risk factors are used for risk stratification of SCD in adult patients. Four clinical risk factors were identified as being ‘major risk factors’ and likely to be associated with SCD in childhood HCM (Table 1): previous aborted cardiac arrest or sustained VT; unexplained syncope; NSVT; and extreme left ventricular hypertrophy. In adults with HCM, syncope occurring within the last 6 months may be more predictive of SCD compared to a more distant syncopal event; the importance of timing of syncopal events has not been explored in childhood HCM
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