2024 Updated Guidelines for Management and Treatment of HCM

The American Heart Association/American College of Cardiology (AHA/ACC) Guidelines for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy published in 2020 have been updated for 2024 to address certain new developments in the field including the addition of myosin inhibitor such as Camzyos (mavacamten) to the treatment arsenal, as well as the benefits of exercise for the HCM population.

The 2024 guidelines update the most recent version published in 2020.  In the past,  guidelines were only updated every decade or so, but with the recognition of major changes in the treatment of HCM with the addition of new drugs such as mavacamten, AHA/ACC wants to keep the guidelines current with emerging developments.  Guidelines are compiled following review and discussion by a committee of HCM experts with reference to the latest published HCM literature.  The document addresses the broad spectrum of issues seen by medical professionals who are called upon to diagnose and treat patients and families affected by hypertrophic cardiomyopathy.

Two patients served on the writing committee for this 2024 update:  Cynthia Burstein Waldman, a HCM patient who is the Founder and Editor of the blog HCMBeat was privileged to sit on the writing committee alongside congenital heart patient and 2020 guidelines committee member Lauren Evanovich and the esteemed panel of medical professionals. Together the committee members discussed the latest research and determined best practices for addressing all facets of HCM.  The inclusion of patients on the committee provides a real world perspective focusing the discussion through the lens and experience of people who have actually lived through and dealt with these and similar medical issues. Lauren and I hope and believe that our input was useful and we are grateful to have played a small part in moving things forward for HCM patients.

Some key updates to the 2024 guidelines include:

  • New Drug Options:  Cardiac myosin inhibitors such as Bristol Myers Squibb’s mavacamten (Camzyos) are now an option to treat patients with obstructive HCM who do not see improvement to their symptoms from first line drug treatments like beta blockers and calcium channel blockers.
  • Exercise:  Exercise is beneficial to patients with HCM and has not been associated with increased risk of ventricular arrhythmias as previously feared and reflected in prior guidelines which urged caution with regard to exercise. Current research reflects the cardiovascular benefit to all, including HCM patients, of moderate exercise.  However, it is critical to seek input from HCM experts and engage in shared decision making about the risks and benefits of exercise before undertaking an exercise program. This discussion should be repeated at regular intervals.
  • Shared Decision Making:  The 2020 and 2024 guidelines emphasize the process of shared decision-making between patients and medical providers.  Medical providers should provide an overview of all testing and treatment options, and engage the patient in a complete discussion of all risks and benefits.  Most importantly, the patient is encouraged to share their own goals, values, and concerns which should inform the final decision.
  • Referral to HCM Specialty Centers Encouraged:  Referral to specialized HCM Centers with expertise in treatment of the disease is important.  Expert advice is especially important where the treatment path is not clearly defined, or for HCM specific procedures and complex treatment and lifestyle decisions.
  • Atrial Fibrillation:  HCM patients with atrial fibrillation, whether it is persistent or paroxysmal (sporadic), have a heightened risk of stroke. Therefore, there should be a low threshold for prescribing anti-coagulant drugs for HCM patients with AFib without regard to traditional stroke risk scoring systems, i.e. the CHADS-VASc score.  Maintenance of sinus rhythm and rate control are key treatment goals.

For key patient takeaway messages from the guidelines click HERE.

For a complete look at the guidelines click HERE.

For a video of the Writing Committee Chair Dr. Steve Ommen and Co-Chair Dr. Carolyn Ho talking about the new guidelines, as well as other and related resources, click HERE.

Vigorous Exercise Safe for HCM Patients

The long awaited results of the LIVE-HCM study have now been published.

HCM previously blogged about the study in March when they were first announced at the American College of Cardiology meetings.

This study followed 1534 HCM patients between the ages of 8 and 60,. Most participated in various forms of exercise over the three year period of the study, such as running, swimming and basketball.  The study also included 126 participants who carried a gene associated with HCM, but did not show overt signs of the disease. 42% of study participants exercised vigorously, 43% exercised moderately, and 16% were not active.

Contrary to an older school of thought which cautioned against vigorous exercise in HCM patients, the results of the study showed NO increase in cardiac arrests, ventricular arrhythmia or fainting among the vigorous exercisers, disproving the long held assumption that vigorous exercise was dangerous for patients with hypertrophic cardiomyopathy.  Also, the study found no cardiac events occurring among the group who only carried a gene for HCM but did not have the disease.

 

Exercise and HCM: Not Enemies Anymore

Lots of good news for active HCM patients was presented at last weekend’s 23rd Annual American College of Cardiology Scientific Sessions.

The long-awaited results of the LIVE-HCM study were presented, and the results were quite reassuring for HCM patients who are vigorous exercisers.

This study followed 1534 HCM patients between the ages of 8 and 60,. Most participated in various forms of exercise over the three year period of the study, such as running, swimming and basketball.  The study also included 126 participants who carried a gene associated with HCM, but did not show overt signs of the disease. 42% of study participants exercised vigorously, 43% exercised moderately, and 16% were not active.

Contrary to an older school of thought which cautioned against vigorous exercise in HCM patients, the results of the study showed NO increase in cardiac arrests, ventricular arrhythmia or fainting among the vigorous exercisers, disproving the long held assumption that vigorous exercise was dangerous for patients with hypertrophic cardiomyopathy.  Also, the study found no cardiac events occurring among the group who only carried a gene for HCM but did not have the disease.

According to Dr. Rachel Lampert of the Yale School of Medicine who was the lead investigator of the study, the data from this study “does not support restriction of vigorous exercise for individuals with HCM.”  Dr. Lampert also spoke about the benefits to health of exercise in general, and the need for shared decision-making between HCM patients and experienced HCM physicians when making decisions around exercise.

You can see this video of Dr. Lampert talking about the LIVE-HCM study.

And in other good news for HCM athletes, another paper supportive of competitive sports in patients with genetic heart disease was presented at ACC23.  This presentation showed that 95% of competitive athletes who played at the Division 1 or professional level who elected to return to play after being diagnosed with a genetic heart disease did not experience cardiac events such as fainting, seizures, ICD shocks, sudden cardiac arrests or sudden cardiac death.  Again, focusing on the need for shared decision-making and risk stratification with an expert, the researchers believe that with a comprehensive return-to-play protocol including a personal AED, annual follow-ups and continued risk evaluation, together with coordination with team and/or organization medical staff, a return to competitive sports may be possible.

Comprehensive risk assessment, combined with shared decision making around the decision to participate in sports, seems to be the way of the future.  It is an important concept that was included and discussed in the 2020 ACC/AHA Guidelines for the Diagnosis and Treatment of HCM and represents an important advance for patients.

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.

ESC Issues New Exercise Guidelines Including Guidance for HCM Patients


The European Society of Cardiology recently published new guidelines for exercise for patients with HCM.

These guidelines offer more flexibility for HCM patients that past guidelines, stating that exercise may be permissible for HCM patients, but that it depends on individual situations. The focus should be on the shared decision process between patient and doctor to determine whether exercise may be appropriate for each individual patient.  Additionally, annual follow up for adults, with more frequent follow up for adolescents and young adults is also recommended.

Section 5.5.1 of the guidelines focuses on Hypertrophic Cardiomyopathy. These guidelines state:

  • The risk of sudden cardiac death or arrest (SCD or SCA) during exercise is likely lower than previously thought and therefore, a total ban on exercise is not warranted.
  • Exercise for physical and psychological well-being should be encouraged, following a careful baseline assessment of patients with HCM which should include:
    • Personal and family history
    • Risk Factors for SCD/SCA
    • EKG at rest and during exercise
    • Echocardiogram/Degree of Obstruction
    • Degree of late gadolinium enhancement indicative of cardiac fibrosis as seen on cardiac MRI
    • Exercise testing and blood pressure response to exercise
    • ESC Score of HCM Risk

 

Overweight HCM Patients Fare Worse

This week, researchers from the eight HCM centers comprising the Sarcomeric Human Cardiomyopathy Registry [SHARE Registry] published a paper that every HCM patient should take to heart.

The sobering findings are that overweight HCM patients have a higher incidence of obstruction, heart failure and atrial fibrillation than their normal weight counterparts. As a result of this study, the researchers suggest heightened attention to weight management and exercise in order to prevent disease-related progression and complications.

Continue reading “Overweight HCM Patients Fare Worse”

The High Hanging Fruit: Treatment for Non-Obstructive HCM – Commentary by Dr. Sharlene Day

A recent study conducted in the U.K. evaluated whether the anti-anginal drug trimetazidine would improve symptoms and exercise capacity for those patients with non-obstructive hypertrophic cardiomyopathy. 

Unfortunately, this study which was conducted by Dr. Perry Elliott and his colleagues at University College London, found that trimetatazidine did not improve exercise capacity in these patients. Following the results of this study, trimetazidine will now join ranolazine and spironolactone in the compost heap of drugs which tried and failed to improve HCM symptoms.  While a third drug, perhexiline, was found to improve symptoms for non-obstructive HCM, its limitations, including potentially serious side effects, stand in the way of its common usage.

In a companion editorial to this study entitled “Non-Obstructive Hypertrophic Cardiomyopathy-the High Hanging Fruit,” Dr. Sharlene Day of the University of Michigan’s HCM Center discusses the difficulties seen in drug trials related to non-obstructive HCM.

Continue reading “The High Hanging Fruit: Treatment for Non-Obstructive HCM – Commentary by Dr. Sharlene Day”

Simple Lifestyle Changes Can Improve HCM Symptoms

This article by Dr. Stephen Heitner of Oregon Health & Science University covers some simple lifestyle changes that can help HCM patients feel much better. In particular, Dr. Heitner mentions:

  • Eating smaller meals and avoiding large carbohydrate rich meals.
  • Avoiding dehydration
  • Limiting alcohol
  • Avoiding exercise after eating
  • Engaging in moderate intensity exercise
  • Managing weight
  • Evaluating and treating sleep apnea and other sleep breathing disorders
  • Getting appropriate treatment for anxiety and depression

The above lifestyle changes, combined with appropriate medical treatment, will keep HCM patients feeling their best.

 

Newest HCM Research Focuses on Exercise

Two articles published this week highlight how attitudes relating to exercise and HCM have changed in recent years.  Older recommendations that HCM patients should refrain from exercise appear to be outdated.

The first article, an editorial published in Circulation by Drs. Sara Saberi and Sharlene Day from the University of Michigan’s HCM Center, reviews the statistics relating to sudden death of athletes and questions the long held notion that participation in vigorous activity or competitive sports is dangerous in HCM.  This editorial also points out that moderate exercise is the only non-surgical intervention proven to increase exercise capacity in patients with HCM.

See this HCMBeat interview with Drs. Saberi and Day for more details about their groundbreaking study.

A second article from researchers in Australia used the number of a patient’s steps per day as an indicator of HCM disease severity and the patient’s functional limitations.

It seems that the time has come for HCM patients to get up off their couches and get moving!

Lifestyle Influences HCM

According to this recent article published in the Journal of the American Heart Association, lifestyle choices can influence the development and/or progression of HCM.

In particular, the authors made the following recommendations:

  •  Exercise:  Recreational exercise should be encouraged in HCM patients.
    • This recommendation was largely based on the findings of the recent RESET-HCM study which found that moderate exercise, specifically tailored to each individual patient’s capacity, was beneficial to the patient’s general health and well being.  **For an in-depth look at the RESET-HCM study, check out this recent HCMBeat interview with the authors of the study, Drs. Sara Saberi and Sharlene Day.
  • Eating and Drinking: 
    • Patients should avoid large meals and should not exercise immediately after eating.  
    • Care should be taken to avoid becoming dehydrated.
    • Alcohol should only be consumed in moderation.
  • Healthy weight should be maintained.
  • Hypertension should be treated aggressively, though treatment may be challenging, especially in obstructive HCM.
  • Obstructive Sleep Apnea, which may exist in as many as 70% of HCM patients, should be treated to minimize potential for arrhythmia and to improve blood flow.