Category: News

Vigorous Exercise Safe for HCM Patients

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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.

 

Weight Loss Improves HCM

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HCM Beat in half circle 3.75

Researchers at New York University’s Langone Health published a recent paper reporting on 6 patients who lost a significant amount of weight (at least 18 lbs.) and who had undergone cardiac MRI or CT scans before and after the weight loss. 

The researchers found that following weight loss, the scans showed a decrease to the dimensions of the left ventricle, and the patients experienced improvement to their symptoms.

The researchers suggest that while more study is needed, weight loss can benefit overweight patients and may be especially helpful to those who have limited treatment options. 

 

Cynthia Burstein Waldman © 2023   

 

Exercise and HCM: Not Enemies Anymore

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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.

Cytokinetics Teases Upcoming Phase 3 Aficamten Trial in nHCM

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A Phase 3 clinical trial aimed at the non-obstructed HCM population (nHCM) appears to be planned for Cytokinetics’ next-generation myosin inhibitor, aficamten.

Based on positive data from Cohort 4 of the Phase 2 REDWOOD-HCM trial, Cytokinetics is planning to soon launch a Phase 3 clinical trial for non-obstructed hypertrophic cardiomyopathy.  Non-obstructed HCM patients are a group whose only current treatment options are diuretics, beta-blockers, and other drugs used in the traditional heart failure setting.  In extreme cases, heart transplantation may be the only path available to these patients.

According to data presented on Sunday at the American College of Cardiology 72nd Annual Scientific Sessions (held in my beloved hometown of New Orleans, the city with the best and least heart-healthy food in the world), 41 patients with nHCM in the cohort showed improvement to both their NT-proBNP and troponin levels (both are biomarkers of heart wall stress seen in blood tests). And, the drug was generally well tolerated.

Separately, data was presented showing that obstructed HCM (HOCM) patients who took aficamten for a 48 week period showed significant improvement to their left ventricular outflow tract gradients (LVOT) while 88% of patients experienced improvement of at least one NYHA Functional Class.

All 19 HOCM patients enrolled in the trial who fit the eligibility criteria for septal reduction therapy (septal myectomy or septal alcohol ablation) at the start of the trial improved so much that they were no longer eligible after 48 weeks on aficamten.

It would be wonderful to have better options for the treatment HCM, so here’s hoping that aficamten continues down the road to FDA approval.

For more about aficamten, read these prior posts from HCMBeat:

2 Companies Testing Drugs for HCM

HCM Clinical Trials – the Latest News

The Future of HCM Care

Positive Signs from REDWOOD-HCM

Promising Data about Aficamten Presented at Meetings

More on Aficamten & Mavacamten from ACC 2022

Aficamten Updates from Cytokinetics

Aficamten Gets “Breakthrough Drug” Status from FDA

Cytokinetic’s Drug Aficamten & Upcoming HCM Summit – Interview with Dr. Martin Maron

Results Published from REDWOOD-HCM Phase 2 Trial

The Future of HCM Treatment

New Technology May Find Early Signs of HCM

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A recent paper published in Nature Reviews Cardiology shows how new advances in Cardiac MRI and biomarkers identified through blood testing may help to identify subclinical HCM before overt signs are evident.

This information could be used to intervene before patients ever develop overt signs of disease. Newer medications or experimental gene therapies have the potential to prevent the condition from ever developing.

These modalities would be useful when screening  relatives of HCM patients and/or in people known to carry a gene for HCM.

Gene Therapy – Is a Cure for HCM Around the Corner?

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Two different papers published in the newest issue of Nature focus on experimental gene therapies to potentially repair the genes that cause hypertrophic cardiomyopathy.  This research even caught the eye of Dr. Eric Topol, a prominent cardiologist at the Scripps Institute whose focus on genetics and digital technologies aims to change the future of medicine.

Researchers in the first study were able to correct pathogenic HCM mutations in an MYH7 gene. This was done both in cardiomyocytes (the cardiac cells which cause the heart to contract) derived from human pluripotent stem cells, as well as in mice with human-like HCM mutations.

The second study used two different genetic therapies to prevent HCM caused by the R403Q mutation in more than 70% of cardiomyocytes carrying the mutation.

The researchers hope that these findings will soon allow scientists to treat patients who carry an HCM gene BEFORE the disease actually develops, thereby interrupting the cycle and preventing the disease

It would be very exciting to see a clinical trial testing this technology. These therapies show great promise for our children and grandchildren.

Echocardiogram of the Future: Size of a Stamp!

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Researchers at UC San Diego have developed a small, wearable ultrasound which uses artificial intelligence (AI) to assess the structure and function of the human heart for up to a 24 hour period.  And most exciting of all, it is only the size of a postage stamp!

Due to its small size and the lack of bulky equipment, the device can be used at home and during vigorous exercise. This capability will allow for the collection of real life data and will simplify testing for patients.

Read the full paper published in Nature here and a short article in Cardiovascular Business here.

Septal Reduction – Not a One Size Fits All

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A recent article published in the European Journal of Cardiology Heart Failure compares septal myectomy to septal alcohol ablation. The paper goes through specific factors which make one procedure or the other more appropriate: individual anatomy and age being two .of the most important.

The article emphasizes that proper patient selection for either procedure is key.  Further, it emphasizes the necessity of having both procedures done at at high volume center in order to increase the odds of a successful procedure.

Seniors Do Well After Myectomy

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A recent study of Medicare patients conducted by doctors at the Cleveland Clinic found that senior citizens who underwent septal myectomy had better long term survival and did not need repeat procedures compared to those who underwent septal alcohol ablation.

And, despite the overwhelming evidence that septal reduction procedures performed at high volume centers have superior outcomes, 70% of septal reduction procedures are still being performed at low volume centers.

We need to spread the word that the success of septal reduction procedures is highly dependent on the experience and volume of the center!

Lisa Marie Presley Dies of Cardiac Arrest at Age 54

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Very sad update to the news about Lisa Marie Presley. RIP. This family has had far too much grief.

HCM Beat

All major news outlets reporting that Lisa Marie Presley has died this afternoon after suffering cardiac arrest at her home this morning.  She was resuscitated at home, and then transported to a local hospital where she passed away.

It has previously been reported that Elvis himself carried a HCM causing gene.   Elvis’ genetic analysis was featured in a program by U.K.’s Channel 4 Television in 2014.  Elvis’ mother also died before age 50 of a “heart attack.”

Sending deepest condolences to Priscilla Presley, daughters Riley Keough, Harper Lockwood and Finley Lockwood.

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