Cytokinetics Moves Forward with HCM Drug Trial

This week Cytokinetics announced that the first patient in Cohort 2 of their REDWOOD-HCM trial has been dosed.

Cytokinetics is testing the safety and tolerability of the cardiac myosin inhibitor CK-274 for the potential treatment of hypertrophic cardiomyopathy.  The trial already enrolled completed enrollment of 18 patients in the first cohort.  Sites are located across North America and Europe and results are expected in the middle of this year.

Separately, Cytokinetics announced that CK-274 has been granted an orphan drug designation by the FDA. This designation confers certain benefits and financial incentives for drugs intended to treat conditions affecting fewer than 200,000 people in the U.S.

iPhone 12 Can Disable ICD!

In this study just published in the Heart Rhythm journal of the Heart Rhythm Society, doctors demonstrated that an Apple iPhone 12 (which is built with more magnets than previous iPhone models due to the Mag Safe charging technology) can disable the ability of an implantable defibrillator to deliver therapy.

So, this is a reminder for ICD patients that according to all device manufacturers, in order to avoid a problem, keep your phone at least 6 inches away from your device, and remember that you should NEVER PUT YOUR PHONE (ANY PHONE, NOT JUST AN IPHONE 12) IN YOUR SHIRT POCKET! 

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Crypts Sign of HCM? Study Says No

According to a recently published study by doctors in Copenhagen, Denmark, myocardial crypts (clefts, cracks or fissures in the myocardium) are found in the general population. Therefore, this article concludes that crypts seen on scans of the heart are not necessarily an indicator of HCM and do not warrant further investigation. 

This paper is a departure from a 2012 paper by doctors at Tufts, which concluded that myocardial crypts were associated with HCM, and that they were often found in relatives of HCM patients found to be gene positive for HCM, but lacking the hallmark thickening of the ventricle. 

Here is an example of what the crypts look like on MRI.

Myectomy Sooner?

An article by doctors at the Cleveland Clinic recently published in the Journal of the American Heart Association advocates for earlier surgical intervention for patients with obstructive hypertrophic cardiomyopathy (HCM).   

According to this article, obstructed HCM patients who undergo myectomy earlier have better long term survival. Therefore, these doctors take the position that patients should not wait until they become severely symptomatic and/or have run out of medical options to undergo myectomy surgery. 

Meanwhile, an accompanying editorial by Dr. Mark Sherrid of NYU Langone Health is to the contrary.  Dr. Sherrid argues that medications like disopyramide (Norpace) are effective in reducing symptoms and that the inherent risks from open heart surgery are not outweighed by a theoretical improvement in longevity.

Regardless of the timing of surgery, Dr. Sherrid points out that with multiple companies now developing novel treatments for HCM, visibility of the disease will increase which will ultimately result in better patient outcomes for all with HCM. 

 

 

 

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”

Positive Signs from REDWOOD-HCM

Cytokinetics announced this week the progression of REDWOOD-HCM, its ongoing Phase 2 clinical trial of CK-274, a cardiac myosin inhibitor for the treatment of HCM.

According to the press release, preliminary results show substantial reductions in left ventricular outflow tract gradients (LVOT), with only modest decreases in left ventricular ejection fraction. (LVEF). 

Based on this early positive data, the study will continue and will enroll a second group of approximately 18 patients. 

Cytokinetics hopes to have results from REDWOOD-HCM in the middle of 2021, and hopes to begin a Phase 3 trial for CK-274 by the end of 2021.

For detailed information about this trial see ClinicalTrials.gov.

More Bad News for Women with HCM

A recent retrospective study by researchers using data from the SHaRe Registry found that women with hypertrophic cardiomyopathy have an increased risk of death and/or severe heart failure symptoms when compared to their male counterparts.  This study also found that women tended to be older when diagnosed with HCM than men. 

This study echoes a 2017 study from Mayo Clinic with similar findings.

Editor’s Note:  It is clear that serious research into gender differences in HCM is needed. And, it is vitally important that women with HCM be persistent and diligent in obtaining expert care for their HCM. Their lives depend on it!

 

Boston Scientific Advisories for S-ICD Patients

Patients with a Boston Scientific S-ICD should be aware of two recent product advisories.
 
 
There is an advisory for early battery depletion in the S-ICD generator, and there is a separate advisory pertaining to the 3501 lead.
 
 
Boston Scientific has a product lookup tool (www.BostonScientific.com/lookup) where you can look up your device and see if your specific generator and/or lead are affected.  You can find your serial numbers on your registration card.
 
 
The product advisories can be found here: https://www.bostonscientific.com/…/product-advisories.html
 

2020 AHA/ACC HCM Diagnosis & Treatment Guidelines Released

The highly anticipated 2020 American Heart Association/American College of Cardiology Guidelines for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy have been released.

This document, drafted with reference to published HCM literature, and with input from a committee of HCM experts with broad expertise, updates the prior version published in 2011.  It contains clinical practice guidelines for the broad spectrum of issues which may confront medical professionals as they approach the diagnosis and treatment of patients and families affected by hypertrophic cardiomyopathy.

Continue reading “2020 AHA/ACC HCM Diagnosis & Treatment Guidelines Released”

MyoKardia Acquired by Bristol Myers Squibb – UPDATED

This morning MyoKardia announced that it was being acquired by drug company Bristol Myers Squibb (BMS) for $13.1 billion. BMS plans to continue MyoKardia’s development of mavacamten as the first drug specifically intended to treat hypertrophic cardiomyopathy. The deal is expected to close before the end of 2020.

Last month, MyoKardia published positive results from its Stage 3 EXPLORER trial for obstructive HCM. Earlier this year, the FDA granted mavacamten breakthrough therapy designation which could shorten the FDA approval process by about 4 months. If all goes well, the drug could be available to HCM patients by the end of 2021.

The merger strengthens BMS’ cardiac offerings. Mavacamten will join Eliquis, a blood thinner that will go generic in 2026.

This is not MyoKardia’s first tie to Big Pharma. The French pharmaceutical company Sanofi invested $230 million into MyoKardia in 2014 but after their agreement expired in 2018, MyoKardia bought back the U.S. rights to its drugs for $80 million.

You can read a press release about the merger from MyoKardia here or a press release from BMS here. You can also read about it in the Wall Street Journal here.

DISCLOSURES:  HCMBEAT HAS RECEIVED UNRESTRICTED EDUCATIONAL GRANTS FROM MYOKARDIA.  ADDITIONALLY, CYNTHIA BURSTEIN WALDMAN OF HCMBEAT SERVED AS A PATIENT ADVISOR ON THE STEERING COMMITTEE OF MYOKARDIA’S EXPLORER TRIAL AND IS CREDITED AS AN AUTHOR OF THE STUDY AS PUBLISHED IN THE LANCET.  CYNTHIA ALSO SERVES ON MYOKARDIA’S PATIENT ADVISORY BOARD.