Arrhythmia Monitoring in HCM

Arrhythmia monitoring in HCM patients is used not only for determining risk of sudden death and potential need for an implantable defibrillator, but also for detecting atrial fibrillation (AFib) and implementing anti-coagulation for stroke prevention.

A recent paper provides an overview of the usefulness of arrhythmia monitoring in patients with HCM. 

THERE ARE DIFFERENT TYPES OF EVENT MONITORS

There are several varieties of event monitor available: some are used for a day or two, some for a couple of weeks, and some implantable devices such as implantable loop recorders can be used to record rhythm abnormalities for several years.

WHEN IS MONITORING APPROPRIATE?

The 2020 AHA/ACC Guidelines recommend arrhythmia monitoring at initial evaluation and then every 1 or 2 years thereafter. Extended monitoring is also recommended for patients with palpitations or lightheaded episodes in order to ascertain and treat the cause.

MONITORING FOR ATRIAL FIBRILLATION

This paper highlights the fact that patients with HCM who have Afib risk factors should undergo an initial screening for AFib and then repeat arrhythmia monitoring every 1 to 2 years thereafter.

IMPORTANT NOTE -The prevalence of AFib in HCM is 4 to 6 times higher than in the general population. Documented AFib of more than 24 hours duration requires lifelong treatment with anti-coagulants in order to avoid a thromboembolic stroke.

MONITORING FOR NON-SUSTAINED VENTRICULAR TACHYCARDIA (NSVT)

Episodes of NSVT found during monitoring, especially when looked at in conjunction with other risk factors, may support the recommendation of an implantable defibrillator.

Worse Exercise Capacity in Women with HCM

According to this recent study looking at exercise capacity in patients with HCM, women with HCM demonstrated reduced exercise capacity when compared to men.  This paper theorizes that the differences are likely attributable to passive diastolic properties and that these could aid in the development of interventions specifically targeted for women.

Aficamten Gets “Breakthrough Drug” Status from FDA

Cytokinetics has announced that its experimental drug aficamten, currently in trials as a potential treatment for obstructive hypertrophic cardiomyopathy, has received breakthrough therapy designation from the FDA.  This designation is awarded by the FDA to certain drugs which may offer substantial improvement to patients over available therapies. The designation could shorten the FDA approval process for the drug by about 4 months.

You can read more about Cytokinetics and aficamten in these older posts from HCMBeat: 

Interview with Dr. Martin Maron about Cytokinetic’s Drug Aficamten 

Cytokinetics Announces Positive Results from REDWOOD-HCM Phase 2 Clinical Trial

Cytokinetics Moves Forward with HCM Drug Trial

Cytokinetics Announces its Phase 2 Clinical Trial

Positive Signs from REDWOOD-HCM

The Future of HCM Care

HCM Clinical Trials – the Latest News

2 Companies Testing Drugs for HCM

Scientists Get $10 Million Grant to Develop HCM Treatments

 

 

FDA Approval of Mavacamten Delayed

The U.S. Food and Drug Administration (FDA) has postponed the date by which it must complete its review of mavacamten – the first drug made specifically to treat HCM. The FDA was originally scheduled to announce its decision in late January 2022, but that date has now been postponed until April 28, 2022, which is the new “PDUFA date” or the date by which the FDA must respond to the new drug application.  

The FDA has not asked Bristol Myers Squibb (BMS) to submit any additional data, but it wants to spend more time reviewing the proposed Risk Evaluation Mitigation Strategy or REMS which is the safety profile of the drug.  The FDA can require REMS for certain drugs with safety concerns to ensure that the benefits of the medication will outweigh any risks.

BMS acquired the drug mavacamten, a first in class myosin inhibitor, through its $13.1 billion acquisition of San Francisco biotech company MyoKardia late last year.

You can read more about mavacamten and its journey in these past entries from HCMBeat:

2 Companies Testing Drugs for HCM

HCM Clinical Trials – the Latest News

HCM Drug Shows Improvement to Heart Structure

More Positive Data on Mavacamten Presented at ACC Meeting

More Positive Results for MyoKardia Drug

MyoKardia Announces Positive Results from EXPLORER Trial

MyoKardia’s EXPLORER Trial Big Success

Mavacamten Approaches Finish Line

BMS Launches New HCM Awareness Campaign Featuring Utah Jazz Player Jared Butler

Bristol Myers Squibb has launched a new hypertrophic cardiomyopathy awareness campaign and website entitled “Could it be HCM?” The campaign launch is in connection with the expected early 2022 FDA approval for the first-in-class cardiac myosin inhibitor drug mavacamten, 

A video made for the campaign features professional basketball player Jared Butler of the Utah Jazz.  In the video, Butler shares his surprise and dismay when he learned of his HCM diagnosis. Butler was fortunate that he was cleared to play basketball by his doctors at the Mayo Clinic who continue to follow him closely.  He was even featured in People Magazine talking about his HCM. See also this article in the Salt Lake Tribune.

The website described what happens to the heart in HCM,  the symptoms of HCM, and provides resources for dealing with a diagnosis of HCM.

Check it out!

HCM News from 2021 AHA Scientific Sessions

This year at the AHA Scientific Sessions several presentations focused on HCM.  A couple receiving attention included:

What Should Mavacamten Cost?

On October 22, patients, physicians, and other interested parties will have the opportunity to provide input on the value and cost of mavacamten – the first drug specifically designed to treat hypertrophic cardiomyopathy. Last year, Bristol Myers Squibb paid $13.1 billion to acquire MyoKardia, the San Francisco biotech company that developed the drug and brought it through clinical trials.

At a virtual public meeting, The Institute for Clinical and Economic Review or ICER will listen to further testimony in order to evaluate mavacamten’s value and potential benefitsICER is a non-profit organization that evaluates the cost effectiveness of drugs and medical procedures. Many insurance companies rely on ICER’s findings when deciding how much to pay for a certain treatment or test. 

In an Effectiveness Report which was published today, ICER valued the benefit that mavacamten would bring to a patient at between $12,000 to $15,000 a year. By contrast, some analysts have suggested that mavacamten could carry a price tag as high as $75,000 per patient per year.

 If you would like to share your thoughts at the online public meeting click here to sign up.  

You can find a press release from ICER about their review of mavacamten here.

HCM Patient Competes on Television Show “The Voice”

Berritt Haynes, a 19 year old with hypertrophic cardiomyopathy, was chosen for Team Blake Shelton last night on the MGM Television/NBC show THE VOICE.  After Berritt’s mother submitted a tape, Berritt was chosen to audition on stage in front of the coaches.

Berritt had hoped to attend a taping of the show last season through Make-A -Wish Foundation which grants wishes to kids with serious health issues. However, due to COVID, he was not able to make that happen.  Instead, this year his mother helped make his dream come true by making it possible for him to actually perform.

Berritt was diagnosed with HCM when he was 8 and received an implantable defibrillator when he was 14.  Clearly, Berritt’s HCM has not interfered with his performing talents.  Watch him performing on The Voice here.

Good luck Berritt. All of the HCM world will be rooting for you to advance to the next round!

Read more at:

Yahoo Entertainment

USA Today

ET Canada

THE VOICE can be seen on NBC on Monday and Tuesday nights. Check your local schedule for times.

Women and Babies Get HCM Too

Recent HCM research has taken a much needed pivot away from a focus on adult men to the exclusion of others. Women and babies get HCM too, yet until this point, there has been far less attention and research on their issues. HCM is not just a disease of adult men, though they do make up the majority of participants in research studies which inform current treatment protocols.

Finally that may be changing.  It seems that people are finally starting to pay some attention to other folks who have HCM.  Here is a recent European article looking at HCM in infants  and here is a recent article looking at HCM in women. 

Let’s hope the trend continues since previous research has shown that HCM is often more severe in these two groups. 

This past article on HCMBeat takes a look at the more severe clinical course of HCM in women while this past article discusses the severity of HCM in children.

New MRI Technology Spots Scar Tissue Using AI

A new technology called “virtual native enhancement” (VNE) may soon eliminate the need for gadolinium as a contrast agent for patients with hypertrophic cardiomyopathy undergoing cardiac MRI. Gadolinium, a heavy metal contrast agent which is injected intravenously, has long been used in cardiac MRI scans to spot cardiac scar tissue in patients with HCM.  In 2017, the FDA issued a safety communication relating to gadolinium  because it was found that gadolinium remains in the body for months to years after the use of the drug.

The new VNE technology, recently described in the journal Circulation, uses artificial intelligence (AI) to virtually enhance the standard MRI image. The technology was developed using data taken from 1348 HCM patients and was validated in the HCM population, but the technology may have uses extending beyond HCM.

By avoiding the use of the contrast agent, this technology avoids side effects and long term consequences from the use of gadolinium. Additionally, it will make cardiac MRI available to patients who are allergic to gadolinium. VNE is also faster and cheaper that current technology used for cardiac MRI, which may make more frequent MRI monitoring of patients feasible.  

To read more about VNE, see also this article in UVA Today, this article in ACM Tech News,  this article in Engineering and Technology, and this article in Science Daily.