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Updated Clinical Guideline for Addressing Hypertrophic Cardiomyopathy Patients

M3 India Newsdesk Jun 14, 2024

Explore the updated clinical guidelines for managing hypertrophic cardiomyopathy, covering innovative treatments, exercise recommendations, and risk assessment for sudden cardiac events alongside insights from leading professional associations.

With the backing of several other professional associations, the American Heart Association (AHA) and the American College of Cardiology (ACC) have published revised recommendations for the care of patients with hypertrophic cardiomyopathy (HCM). The paper includes revised guidelines on cardiac myosin inhibitors, physical exercise, and risk stratification for sudden cardiac death, with a distinct section for paediatric patients. It does this by combining the most recent information with an emphasis on collaborative decision-making.

Hypertrophic cardiomyopathy (HCM) is a hereditary cardiac disorder mostly brought on by a genetic defect that causes the heart muscle to grow excessively (hypertrophy), impairing the heart's capacity to circulate blood throughout the body. About one in every 500 persons are afflicted with HCM; however, many instances go misdiagnosed since few patient symptoms are present. Sometimes, after an unexpected death, HCM is discovered for the first time. In those who do exhibit symptoms, fainting spells, chest discomfort, dyspnea, or erratic heartbeats are possible.

The revision is the first to adopt a novel approach where portions with fresh data are updated rather than a whole rewriting of the guidelines. The April 2022 approval of mavacamten by the US Food and Drug Administration for the treatment of symptomatic obstructive HCM prompted this most recent edition.

These recommendations help patients with hypertrophic cardiomyopathy live active, healthy lives and clarify the role that cardiac myosin inhibitor medication now plays in patient care.

The American Medical Society for Sports Medicine, the Heart Rhythm Society, the Paediatric & Congenital Electrophysiology Society, and the Society for Cardiovascular Magnetic Resonance collaborated in the development of the guideline, which was published in Circulation and The Journal of the American College of Cardiology.

Inclusion of mavacamten

  1. When first-line medications are neither working nor being tolerated by individuals with symptomatic obstructive heart failure (HFM), the paper recommends using cardiac myosin inhibitors as a class 1 treatment.
  2. They now have the opportunity to give patients that they can prescribe for them right there in the clinic in locations without surgeons or interventional cardiologists to perform the invasive treatments.
  3. However, not all patients should choose this course of action, according to some doctors. In actuality, mavacamten doesn't relieve symptoms for around one-third of people.
  4. Therefore, it's not like it completely eliminates the necessity for those other surgeries, and many people prefer to have a remedy for an anatomic issue rather than take medicine for the rest of their lives.
  5. Experts also noted that many people find the risk evaluation and mitigation strategy (REMS) programme, which calls for regular echocardiographic assessments in order to prescribe mavacamten, to be off-putting.
  6. A lot of planning and scheduling goes into monitoring to ensure that the medication has no side effects, which makes some patients and healthcare professionals less excited about taking it.

Explanations regarding exercise

An "evolution" in how HCM patients should approach physical exercise is another significant addition to the recommendations. It was previously advised to limit exercise to low-to-moderate intensity.

We now know that most patients may safely engage in "vigorous" exercise, which is defined as at least an hour of activity per week that exceeds the threshold of 6 METS, such as brisk running. This received a recommendation of class 2a. Therefore, it's not as strenuous as professional athletes. It's intense in comparison to what we expect everyone attempting to enhance their physical activity and health would be doing as part of a healthy lifestyle.

Nevertheless, guidelines for physical activity at the elite level of sports competition remained unaltered. We just believe that published research hasn't addressed the intensity of training, competing conditions, etc., thus it's still a class 2b. Most importantly, especially those without symptoms, doctors can now advise patients that they should be exercising at the same intensity as everyone else.

Added features

  1. The HCM recommendations now include a dedicated section for paediatric patients since new risk-prediction methods have been developed recently for this demographic. However, there was also a logistical component to this distinction.
  2. The document appears to be updated by designating a distinct category for children. Apart from these new instruments and danger flags, everything was there before. It's now a little more stand-alone than it was for adults, which may help practising paediatric cardiologists pay it more attention.
  3. A class 1 guideline for more extensive, rigorous surveillance for subclinical atrial fibrillation is another significant modification. Patients with a greater risk of A-fib have a risk assessment aim.
  4. The purpose of that approach is to monitor patients more regularly with an ambulatory ECG to ensure that we're not leaving them vulnerable to stroke risk.
  5. One of the most significant unanswered questions in this subject may be how to manage symptomatic nonobstructive HCM.
  6. Numerous experiments are being conducted to investigate it, but we don't currently have enough information to include in the guideline paper.
  7. More improvement of risk-prediction models in HCM is still required. Within the next several years, he anticipates that continuing clinical research in these fields will allow for another update to the guidelines.

Key messages

  1. Shared Decision-Making (SDM) helps people with heart health disorders like HCM achieve better health outcomes. This technique involves the patient sharing treatment objectives and preferences and the physician suggesting the best solutions. This helps patients understand how proposed therapies and lifestyle changes may influence their health, and doctors may recommend treatments that match their requirements and preferences.
  2. Encourage HCM patients to seek care at a multidisciplinary centre for difficult treatments and lifestyle choices. Multidisciplinary centres provide diagnostic tests, innovative therapies, surgeries, and tailored lifestyle and treatment options for HCM patients. Patients usually do better in specialist centres.
  3. Most HCM patients benefit from exercise. Due to the risk of abrupt cardiac mortality, HCM patients were discouraged from intense activity. The new study suggests exercise is safe and good for adolescents and adults, although doctor advice is necessary before starting a programme. Understand that exercise improves heart health in both HCM and non-HCM individuals.
  4. Recent years have seen the development of cardiac myosin inhibitors, a novel medication class for treating obstructive HCM. FDA-approved medication Mavacamten. This new treatment may replace existing prescriptions or invasive procedures for certain individuals. This medication reduces cardiac muscle spasms that block. Patients using this drug must be monitored. Shared decision-making helps eligible patients understand their treatment choices, how they will influence their health, and if they meet their treatment objectives and preferences.
  5. Patients with HCM must understand the danger of Atrial Fibrillation (AFib) and stroke. HCM patients are more likely to develop Atrial Fibrillation. In AFib, the heart pumps blood abnormally. Due to poor circulation, blood may pool in the heart and form clots. These clots may block brain blood arteries, causing stroke. Any episode of atrial fibrillation should be followed by a therapeutic decision-making session with your doctor.


Disclaimer- The views and opinions expressed in this article are those of the author and do not necessarily reflect the official policy or position of M3 India.

About the author of this article: Dr Monish Raut is a practising super specialist from New Delhi.

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