Myocardial Fibrosis in Hypertrophic Cardiomyopathy: What Remains to be Proven?

Hypertrophic cardiomyopathy (HCM) is a complex and well-characterized disease. Its prevalence in the general population is 1 in 500 individuals, with some data suggesting an even higher prevalence when using associated genetic criteria. This means that, based on a conservative estimate, cities such as Goiânia-GO or Recife-PE have approximately more than 3,000 individuals with the disease, a figure that can be as high as 25,000 individuals in a large city such as São Paulo.

Although HCM is perceived as a rare and usually very severe disease, related to dramatic episodes of sudden death (SD) in young individuals and athletes, it is a disease with low mortality. Due to advances in the diagnosis, treatment and prevention of SD, current estimates indicate a mortality rate of 0.5% a year, similar to that found in the general population, with most patients with the disease being asymptomatic or minimally symptomatic. In this scenario, the correct identification of subgroups of individuals with a higher risk of unfavorable outcome and, therefore, a greater chance of benefit when submitted to specific therapeutic strategies, such as the use of implantable cardioverter defibrillators (ICD), is of utmost importance.

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