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.

Picture Improved for HCM Patients with Afib

According to a recent overview by HCM specialists at the Lahey Clinic, the prognosis for patients with hypertrophic cardiomyopathy and atrial fibrillation has substantially improved in recent years.

The use of direct anti-coagulant drugs like Pradexa, Xarelto and Eliquis, which began in 2010, has had a major impact in reducing stroke risk in HCM patients who suffer from Afib.  And, there is a low threshold for the initiation of these drugs for HCM patients who experience episodes of atrial fibrillation, even if such episodes are sporadic or infrequent.  Also, anti-arrhythmic drugs as well as catheter and surgical ablation procedures can be helpful treatment options which are available to these patients.

There are also behavior modifications which can have a positive impact on Afib since patients who are overweight or who suffer from obstructive sleep apnea have a higher risk for atrial fibrillation.

Featured below are some past articles on HCMBeat about atrial fibrillation:

Atrial Fibrillation May Cause Negative Outcomes in HCM

Atrial Fibrillation? Try Giving Up Alcohol

Atrial Fibrillation: Treatable HCM Complication

Better Screening and Treatment Necessary for Atrial Fibrillation

Can This Formula Predict AFib in HCM Patients?

LGE Seen on MRI Top Risk Factor for HCM

To date, risk assessment in patients with hypertrophic cardiomyopathy (HCM) has largely focused on whether there is a need for an implantable defibrillator by evaluating an individual patient’s short term risk of sudden death, but has not looked at the long term risks of heart failure and other adverse outcomes over time.

A recent retrospective study by European HCM specialists asked the question of whether the addition of advanced imaging could assist in pinpointing patients at high risk of heart failure and/or other serious HCM complications over time. 

Continue reading “LGE Seen on MRI Top Risk Factor for HCM”

Recommendations for HCM Depart from 2020 ACC/AHA Guidelines

A recent article by several noted HCM experts shares their recommendations for the screening and evaluation of patients with HCM using several scenarios commonly encountered in clinical practice. Notably, this paper makes several recommendations which differ from the 2020 ACC/AHA Guidelines for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy (2020 Guidelines).

In particular, the article recommends:

  • Cardiac MRI with contrast upon initial evaluation, and every 3 – 5 years thereafter.  (The 2020 Guidelines recommend Cardiac MRI in certain situations, but not for all patients)
  • Genetic testing only in cases where a pathogenic or likely pathogenic mutation has been identified in a family member and in limited other circumstances (the 2020 Guidelines recommends genetic testing for all as part of the initial assessment)
  • Screening of family members 12 or older only (the 2020 Guidelines recommend screening of children of any age when genetic HCM is diagnosed in a family member and no later than puberty, in any event)
  • That HCM patients be disqualified from competitive sports. (The 2020 guidelines instead recommend a shared decision making approach).

Continue reading “Recommendations for HCM Depart from 2020 ACC/AHA Guidelines”

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. 

Continue reading “Arrhythmia Monitoring in HCM”

Can Cardiac MRI Predict AFib in HCM Patients?

In a recent study, researchers examined whether cardiac MRI results might help predict which patients would would go on to develop atrial fibrillation (AFib) that was serious enough to require hospitalization, require electrical cardioversion or catheter ablation, or identify those patients who might go on to develop permanent AFib.

The study found that the major predictors of these serious AFib consequences in HCM were those who were of older age, those with an increased BMI (this was especially important in patients under age 33), increased left atrial volume index as seen on cardiac MRI (this was especially important in middle-aged patients), reduced left atrial contractile function (this was especially important in middle-age and older patients), and moderate or severe mitral valve regurgitation. 

The researchers concluded that using cardiac MRI to measure left atrial volume and contractile function might help medical providers ability to intervene before major AFib develops in HCM patients, specifically by helping patients find ways to reduce their weight and by treating mitral regurgitation and left atrial function more aggressively.

The full paper can be found here and for a summary from Cardiac Rhythm News click here.

 

Can This Formula Predict AFib in HCM Patients?

HCM specialists at Tufts Medical Center and Toronto General Hospital have devised a formula which they hope will help predict which HCM patients may go on to develop atrial fibrillation (“AFib”) over time. This tool can assist doctors in determining which patients are at highest risk so that these patients can be closely monitored and treated appropriately. AFib can be extremely dangerous for HCM patients since it can precipitate a stroke if not appropriately treated.   

Because existing tools to predict atrial fibrillation have not proven to be accurate for HCM patients, the researchers studied 1900 HCM patients with the goal of devising a new tool to help HCM patients and their physicians learn their personal risk for AFib over a 2 and 5 year period.

Continue reading “Can This Formula Predict AFib in HCM Patients?”

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. 

Continue reading “HCM Treatment: The View from OHSU”

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.

Atrial Fibrillation? Try Giving Up Alcohol

A recent study published in the New England Journal of Medicine found that drinkers suffering from atrial fibrillation who stopped drinking for the period of the study significantly reduced episodes of atrial fibrillation. 

According to doctors, alcohol consumption appears to be a significant risk factor and trigger for atrial fibrillation, while teetotaling appears to have a profound impact.

Dr. John Osborne, an American Heart Association spokesperson, said the benefit from giving up drinking was similar to results seen from drugs used to treat atrial fibrillation.  Even if patients are not able to completely abstain from alcohol, Osborne advised cutting back significantly. “It costs nothing and led to a substantial reduction in hospital rates. People in the abstinence group also lost an average of 3.8 kilograms [8.4 pounds] in six months,” he said.

Not everyone thinks that teetotaling is a workable treatment for afib, however. Critics say that encouraging abstinence is unrealistic and is not a permanent solution to the problem.  In fact, a planned follow-up study had to be shortened due the difficulty of finding participants willing to abstain from drinking for a whole year.

 

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