Hypertrophic Cardiomyopathy

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Background:Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiac disorders, with a prevalence in young adults of one in 500 and is defined by its wall thickening.Methods:This chapter of the supplement will present major clinical impacts of this disorder in its predilection to be inherited; its reputation as the most common cause of sudden death in young, healthy, athletic individuals; and its potential to develop heart failure due to either diastolic or systolic dysfunction, so-called “burned out” HCM. Underlying etiologies; diversity of morphologic, functional, and clinical features; and variable age of onset that differentiate the childhood from the adult form of disease will be discussed based on the literature and clinical experience.Results:In children less than 1 year of age, hypertrophy associated with systolic dysfunction is common. In contradistinction, among apparently healthy young adults, the prevalence of echcocardiographically defined HCM was reported to be as high as 0.2% and associated with diastolic dysfunction. In addition, overlaping disorders such as infiltrative and energy-dependent forms of HCM coexist with other atypical features in childhood, further confounding the presentations, treatments, and outcomes compared to adult disease.Conclusion:HCM in childhood has a variety of etiologies which may influence diagnostic testing, treatments, and outcomes.(PACE 2009; 32:S23–S31)

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