This study looked at 4893 patients with hypertrophic cardiomyopathy treated at 7 different European HCM centers between 1980 and 2013. Although the statistics improved for those who were treated more recently, this study makes it clear that there is still much room for improvement in risk stratification and treatment for patients with HCM.
Here is an informative new video from our friend Doug Rachac that nicely explains the safety of MRIs for patients with implantable defibrillators and pacemakers.
I wrote a blog piece about this same issue a few years back. Here it is:
And a few other relevant blog entries here on HCMBeat:
Last year, Doug wrote this blog entry for HCMBeat specifically about magnets and airports. Read that here:
And, you can find more about ICDs from Doug on his YouTube Channel.
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.
Several years ago, researchers from the University of Virginia (UVA) and the University of Oxford announced a joint project involving a large international registry of hypertrophic cardiomyopathy (HCM) patients to facilitate research into HCM. Backed with funding from the National Heart Lung and Blood Institute, this project, known as the HCM Registry, includes data from 2,750 patients with HCM at 44 sites in six countries.
This week, researchers from UVA announced their first findings from this registry which suggest that HCM patients can be separated into two basic groups:
- Patients with a known genetic mutation who are not obstructed but have scarring of the heart muscle;
- Patients who do not have a known genetic mutation and do not exhibit scarring, but who do have a significant amount of obstruction to blood flow.
According to Dr. Christopher Kramer of UVA, this information should provide doctors with information that allows them to better assess the degree of risk to any particular patient, and to help inform a treatment strategy for each patient based on his or her unique profile.
This Medscape article highlights the extraordinary efforts of Dr. Harry Lever, Director of the Cleveland Clinic’s Hypertrophic Cardiomyopathy Center, in educating patients and physicians alike about quality issues with generic drugs. Dr. Lever has been instrumental in publicizing the fact that generic drugs are NOT always the same as their name brand counterparts, and that foreign generics are not put through the same level of scrutiny as drugs in the U.S.
A recent study by Canadian researchers published in the American Heart Association journal Circulation found a much lower incidence of HCM related sudden death than expected. The study included deaths in individuals that occurred between the ages of 10 and 45 in the Canadian province of Ontario between 2005 and 2016. According to lead author Dr. Paul Dorian of the University of Toronto, the expectation was that 1 incident of sudden cardiac death would be identified in every 100 to 200 people who had HCM. Instead, researchers found that the likelihood of sudden death of HCM patients was instead only about 1 in 3,000 people/year.
The study also found that found 7 in 10 HCM-related sudden deaths occurred in people not previously diagnosed with the condition. Men had more than 5 times the risk of sudden cardiac death than women, and most deaths occurred during rest or light activity: only about 17% happened during or immediately after exercise.
Another clinical take away from the study, according to the researchers, is that current exercise guidelines for HCM patients may be too restrictive.
NEWS FROM MYOKARDIA
MyoKardia recently announced a new clinical trial of its drug, mavacamten (formerly known as MYK-461) which will compare the clinical results of mavacamten with septal reduction therapies currently used in clinical practice, i.e. the open heart surgical procedure known as septal myectomy and the catheter based procedure known as alcohol septal ablation.
The study will be run by the Cleveland Clinic with Dr. Milind Desai serving as principal investigator. MyoKardia expect to begin enrolling patients in early 2020.
Read the press release here.
NEWS FROM CYTOKINETICS:
Cytokinetics released positive data from its Phase 1 study of the drug currently known as CK-274 in a poster session at the HFSA 23rd Annual Scientific Meeting in Philadelphia. The study found that CK-274 was safe and well tolerated, while no serious adverse events or negative changes to vital signs, ECGs or laboratory tests were observed.
The company will now be moving into a Phase 2 clinical trial of CK-274 in patients with obstructive hypertrophic cardiomyopathy, expected to begin in late 2019.
Read their press release here.
DISCLOSURES: HCMBEAT HAS RECEIVED PAST UNRESTRICTED EDUCATIONAL GRANTS FROM MYOKARDIA. ADDITIONALLY, CYNTHIA BURSTEIN WALDMAN OF HCMBEAT SERVES AS A PATIENT ADVISOR ON THE STEERING COMMITTEE FOR MYOKARDIA’S EXPLORER TRIAL.
Recently, Cynthia Waldman of HCMBeat corresponded with Dr. Srihari S. Naidu of Westchester Medical Center the second edition of an HCM textbook he recently edited, as well as about medical education surrounding hypertrophic cardiomyopathy in general. What follows is a transcript of their correspondence (which has been slightly edited for readability).
Updated with recent data on mavacamten from ESC Paris, 2019.
Two San Francisco based companies are now conducting clinical trials for three drugs specifically targeting HCM.
MyoKardia, which was founded in 2012 by a group of HCM researchers (including Stanford’s James Spudich, one of the founders of Cytokinetics – the second company conducting a HCM drug trial – see below), was the first entrant into the HCM area with the development of its drug, mavacamten (formerly known as MYK-461).
Mavacamten is currently the subject of the Phase 3 EXPLORER-HCM clinical trial for obstructive HCM, now fully enrolled with results expected in 2020, as well as the Phase 2 MAVERICK-HCM trial for non-obstructive HCM, with results are expected later this year.
And, MyoKardia announced this week that it is will begin testing a second drug for HCM. The new drug, currently known as MYK-224, is the subject of a new Phase 1 clinical trial. This drug targets the sarcomeric…
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