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Invited Commentary
May 22, 2019

Sudden Cardiac Death Risk in Hypertrophic Cardiomyopathy: Wither Our Cognitive Miser

Author Affiliations
  • 1Mayo Hypertrophic Cardiomyopathy Clinic, Mayo Clinic, Rochester, Minnesota
JAMA Cardiol. Published online May 22, 2019. doi:10.1001/jamacardio.2019.1438

Imagine being told that you have been diagnosed with hypertrophic cardiomyopathy (HCM) and that among the things you need to worry about are the facts that your children may inherit this and that you may be at increased risk for dying without warning via sudden cardiac death (SCD).

“What do you mean? I can die suddenly? How likely is it? Can we prevent it?”

In this issue, Maron et al1 report on their experience wrestling with this discussion in more than 2000 consecutive patients. Using what amounts to an evolution of the model introduced in the 2011 American College of Cardiology Foundation/American Heart Association HCM guidelines,2 these data show a strong ability to distinguish those who went on to experience an SCD event in the subsequent 5 years from those who did not. They report a sensitivity of 95% and number needed to treat of only 6.4. Why is this important? Certainly, the emergence of new risk markers since that guideline was published is an important issue, but there is another important undercurrent at play. This is the collateral damage from the ongoing battle between the healer and the cognitive miser.

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