Crypts Sign of HCM? Study Says No

According to a recently published study by doctors in Copenhagen, Denmark, myocardial crypts (clefts, cracks or fissures in the myocardium) are found in the general population. Therefore, this article concludes that crypts seen on scans of the heart are not necessarily an indicator of HCM and do not warrant further investigation. 

This paper is a departure from a 2012 paper by doctors at Tufts, which concluded that myocardial crypts were associated with HCM, and that they were often found in relatives of HCM patients found to be gene positive for HCM, but lacking the hallmark thickening of the ventricle. 

Here is an example of what the crypts look like on MRI.

HCM Treatment: The View from OHSU

If you are looking for a good survey of current practices in the treatment of HCM, a recent article published in the journal Structural Heart by Dr. Ahmad Masri and the team at Oregon Health and Sciences University (OHSU) provides an informative overview of thirty controversies and considerations in the treatment of HCM. This article explains in some detail how the doctors at this HCM Center approach these situations. 

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More Bad News for Women with HCM

A recent retrospective study by researchers using data from the SHaRe Registry found that women with hypertrophic cardiomyopathy have an increased risk of death and/or severe heart failure symptoms when compared to their male counterparts.  This study also found that women tended to be older when diagnosed with HCM than men. 

This study echoes a 2017 study from Mayo Clinic with similar findings.

Editor’s Note:  It is clear that serious research into gender differences in HCM is needed. And, it is vitally important that women with HCM be persistent and diligent in obtaining expert care for their HCM. Their lives depend on it!

 

Atrial Fibrillation May Cause Negative Outcomes in HCM

 

A recent study published in Clinical Research in Cardiology  found that atrial fibrillation is a frequent complication of HCM and can be an important predictor of negative outcomes.

These findings suggest that practitioners should aggressively screen for and treat atrial fibrillation in HCM patients to improve long term outcomes.

Share Registry Study Focuses on So-Called “End Stage” HCM

Researchers from the Sarcomere Human Cardiomyopathy Registry (or the acronym “SHaRe” for short) have again joined forces to look at a subset of hypertrophic cardiomyopathy (HCM) patients who have what is known as “end stage” HCM.  This term, which is a bit of a misnomer, is used to describe patients who experience left ventricular systolic dysfunction (LVSD) which occurs when the left ventricular ejection fraction (LVEF) is less than 50%.

According to the study published last week in the journal Circulation, of a total of 6,793 HCM patients included in the study, 553 or 8% fell into this category.   The paper goes on to highlight some important observations about patients that fall into this category which are highlighted below.

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Coronavirus Guidelines for HCM Patients

In consultation with numerous HCM specialists across the U.S., the Hypertrophic Cardiomyopathy Association has compiled a document of guidelines for HCM patients to follow during the current coronavirus crisis.

Some of the specific recommendations are as follows:

  1. Patient with class 3 or 4 heart failure – specifically those with depressed ejection fractions and those awaiting transplantation – should be advised to stay close to home and minimize exposure in social gatherings.
  2. All patients should be encouraged to practice good personal hygiene including frequent hand washing and to use of disinfectants on all surfaces, doorknobs and banisters.
  3. If a HCM patient tests positive for the virus, they should contact their HCM specialist immediately in order to coordinate care with their local physicians and HCM team.

You can find the whole document here.

Additionally, the HCMA has recorded a series of informative webinars with several
HCM experts which you can find here.

Could Artificial Intelligence Be Useful for HCM Screening?

A study by researchers from Mayo Clinic published this week in the Journal of the American College of Cardiology found that an artificial intelligence algorithm was able to detect hypertrophic cardiomyopathy, commonly known as HCM, from EKG results with impressive accuracy, particularly among younger patients.

In order to “teach” the computer, the researchers used digital 12-lead ECGs from 2,448 patients with HCM along with 51,153 age- and sex-matched controls. The technology was then tested on 612 HCM patients and 12,788 controls.

The findings showed that the technology was able to identify HCM in a high number of cases, even where the EKG appeared “normal” to the human eye.

The researchers believe that this technology, when refined, may prove to be an efficient tool for HCM screening in the future. The team plans to continue testing the technology in greater subject samples in order to further refine its performance.

See also:

Mayo Clinic News Network

Health Analytics

 

AHA Seeking Patient Input for New HCM Initiative

In November at the American Heart Association’s Scientific Sessions held in Philadelphia, AHA announced that it would be starting a three-year initiative focused on hypertrophic cardiomyopathy, also known as HCM.  The initiative is sponsored by MyoKardia, a San Francisco based company that is currently in clinical trials for mavacamten, the first drug specifically intended to treat HCM.

Last week, Cynthia Waldman of HCMBeat had the opportunity to speak with Amy Schmitz, AHA’s National Corporate Relations Director and Alexson Calahan, a Communication Manager for AHA.

What follows is a summary of their conversation about the forthcoming HCM initiative that has been edited for clarity.

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Biventricular Pacemaker For Non-Obstructed HCM Patients?

A small study of  29 patients conducted recently in the U.K. found that the use of a biventricular pacing  in patients with non-obstructive hypertrophic cardiomyopathy improved symptoms of breathlessness and improved exercise capacity as demonstrated during oxygen consumption testing.

Medications are the only treatments currently available to non-obstructed patients. The authors of this study hypothesized that biventricular pacing could be a viable way to address exercise limitations in non-obstructed patients if medications have been ineffective.

Larger trials may establish biventricular pacing as a viable treatment for non-obstructed patients in the future.

The Future of HCM Care

Dr. Stephen Heitner, together with his colleagues at Oregon Health & Sciences University, published an article last week in the European Journal of Heart Failure which gives a glimpse into the treatment of hypertrophic cardiomyopathy (HCM) in the future.  Although recent publications have stated that the majority of HCM patients today have a favorable prognosis when receiving appropriate treatment, a heavy disease burden continues to be placed upon patients.  Hence, better and more effective treatments for HCM are still needed in order to lessen this burden.

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