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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The August 16, 2018 online version of the New England Journal of Medicine contains an broad overview of the current state of clinical knowledge and treatment of HCM written by HCM expert Dr. Barry Maron. It is entitled “Clinical Course and Management of Hypertrophic Cardiomyopathy.”
Dr. Maron discusses the many advances that have been made in the diagnosis and treatment of hypertrophic cardiomyopathy since it was first described 55 years ago, noting that life expectancy and qualify of life have dramatically improved in this period of time. According to Dr. Maron, the contemporary management paradigm for HCM have reduced “the risk of adverse cardiovascular events and death to levels below the levels among patients with other cardiac or non-cardiac disorders.”
As of July, 2018 the HCMCare.com website and app have been updated to include even more information for HCM patients. Check it out.
NOTE: As of July, 2018 the website and app have been updated to include even more information for HCM patients. Check it out.
Have you heard that there is a new online educational resource about HCM? Check out HCM Care.com, an informational website about HCM developed by MyoKardia in partnership with Duke Clinical Research Institute.
This website features general information about HCM including diagnosis, testing, treatment, lifestyle, genetics and family screening. It is also available as a FREE downloadable app for both Apple and Android operating systems.
- Dr. James Daubert from Duke University Medical Center
- Dr. Milind Desai from Cleveland Clinic
- Dr. Carolyn Ho from Brigham and Women’s Hospital
- Dr. Martin Maron from Tufts Medical Center
- Dr. Andrew Wang from Duke University Medical Center
Be sure to check out HCMCare, as…
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According to several news reports, CNN chief and former NBCUniversal head Jeff Zucker is taking six weeks off to undergo elective surgery to treat his hypertrophic cardiomyopathy. Specific details about the surgery were not revealed. New York Magazine reported that in 2010 he visited Minneapolis Heart Institute where he was told he needed an implantable defibrillator.
The most common surgery for the treatment of HCM symptoms is a septal myectomy.
See these stories for more info:
HCMBeat wishes Mr. Zucker the best of luck during his surgery and recovery.
Here is a link to some resources we have collected for patients who are going through myectomy: Resources for Patients About Myectomy
Yet another study has confirmed the safety of MRIs in patients with non-MRI conditional ICDs and pacemakers.
The latest study, from the University of Pennsylvania and published in the New England Journal of Medicine, found no adverse effects from 1.5 Tesla MRI scans performed on 1509 patients who underwent a total of 2103 scans. 58% had pacemakers and 42% had ICDs.
You can also read my personal experience trying to get a brain MRI in Los Angeles last year here.
Updated to include editorial by Dr. Paolo Spirito in recent issue of Circulation.
This study found that AF was not a frequent cause of death by heart failure or sudden cardiac arrest. However, the researchers identified AF as an important cause of stroke in HCM patients. Therefore, they recommend a low threshold for starting HCM patients on anti-coagulants following an initial AF episode.
Researchers in this study analyzed statistics from 1558 HCM patients, 20% of whom experienced AF. 74% experienced only sporadic episodes, while 26% went on to develop permanent AF.
At the time of publication, 91% of the 277 of the patients included in the sample were still alive and between the ages 49 and 75 years old.
According to an accompanying editorial by Italian HCM expert Dr. Paolo Spirito, the outlook for HCM…
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Updated to include a link to a BBC interview with Miles Frost’s brother Wilfred about his brother and father’s deaths from HCM.
Pippa Middleton, (sister of Dutchess Katherine of Cambridge and sister-in-law of Prince William) and her brand new husband, James Matthews, made their first public appearance as a married couple at a fundraiser for the Miles Frost Fund. The Frost Fund, founded in memory of Middleton’s late friend Miles Frost who died from undiagnosed HCM, raises money for genetic testing of family members who have lost a close relative to sudden cardiac arrest, as well as funding HCM research.
Pippa, just back from her honeymoon to French Polynesia and Australia, looked refreshed in her white jumper and carried a red heart-shaped clutch to emphasize the purpose of the occasion.
The fundraiser also attracted other royals such as Princess Eugenie and Sarah Ferguson, Duchess of York.
Here is a video of Wilfred Frost, brother of Miles Frost and son of Sir David Frost, talking about his father and brother’s deaths and the formation…
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A recent study of U.K. HCM patients compared a racially mixed sample of 425 patients, including 163 black patients and 262 Caucasians. The study concluded that while asymmetric septal hypertrophy was the predominant pattern in both ethnic groups, black patients had more instances of apical and concentric hypertrophy, which could, in turn, be responsible for delayed diagnosis of these patients.