Different Treatment for Non-Genetic Hypertrophic Cardiomyopathy?

Many HCM patients, perhaps even the majority, are currently unable to identify the specific gene behind their HCM through genetic testing.  Despite this obvious difference, family screening, risk stratification and treatment standards are no different for patients who carry a HCM gene and those who do not have identified gene(s).

A recent article published in Circulation suggests that there ARE differences which should result in different treatment for this subset of patients.

In particular, non-sarcomere positive patients:

  • have a better prognosis, with lower rates of heart failure, sudden death, atrial fibrillation and stroke
  • Have lower incidence of family members affected by the disease
  • Are more likely to have additional medical conditions such as obesity, hypertension and diabetes

The article by Dr. Hugh Watkins, a British HCM and genetic expert, suggests that:

  • the risk to first degree relatives of this type of HCM patient is less than 50% and therefore, there is less need for repeated screening of relatives
  • Hypertension should be treated more aggressively in these patients.

Read more about non-genetic HCM here and more about screening these patients here on HCMBeat.

3 thoughts on “Different Treatment for Non-Genetic Hypertrophic Cardiomyopathy?

  1. Dear Cynthia

    Your contributions and mailings are so useful, I don’t thank you for every one of them but please be assured that I really appreciate them!

    I am still struggling with HCM definitions and prevalence, in view of the conflicting numbers 1:500, 1:2000, 1:3.500 all very serious articles. We have the iceberg under water, and we have the false positives. And of course,do we count the light phenotypes as patients?

    So it remains very obscure to me and I wonder, why do they call this cohort sarcomere negative? They cannot know that, because so many genes are unknown. As yet.

    Or can these indeed, should they be sarcomere negative, be false positives, i.e. hypertrophy but incorrectly diagnosed? The more we know, the less we know.

    Keep up the good work! I am cheering you on. Now that Myokardia has been absorbed, are you still on that board as patients rep? I hope the board still exists…. they are rolling up offices. The Myokardia Dutch office is now closed.

    Best wishes!

    mARION

    Op 19-8-2021 om 19:38 schreef HCM Beat: > WordPress.com > hcmbeat posted: “Many HCM patients, perhaps even the majority, are > currently unable to identify the specific gene behind their HCM > through genetic testing. Despite this obvious difference, family > screening, risk stratification and treatment standards are no > different for” >

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  2. I am an HOCM/HCM patient with no genes identified as the causative factor in my disease. Sometimes I feel like a second class citizen because the majority of new drugs are tested on those with specific genetic disorders, even though 50% of HCMers have not had a genetic variant identified.
    My Cardiologist DID just send me an article from the New England Journal of Medicine from 9/14/21 ( I think) that shows a significant reduction in hospital admissions and death with the use of Jardiance in patients with Heart Failure with preserved EF….which many of us have! The proverbial Stiff Heart. I’ll let you know if it helps with my symptoms.

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    1. Jill, so are you trying Jardiance now? I’ve been looking for a study of this drug in HCM but I haven’t found anything beyond the one for its use in HFpEF. Seems like an area worth study for a potential new indication for this class of drug.

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