A retrospective analysis recently published in JACC: Cardiovascular Interventions suggests that the risks of septal reduction therapy may differ for men and women.
Are you a HCM patient that is interested in sharing your insights on new patient education resources?
The American Heart Association is recruiting patients to participant in several focus groups sessions that are underway now. If you are interested, please complete this brief pre-screening survey and AHA staff will follow up with you if you are eligible to participate.
AHA is offering gift cards for those who participate in these focus groups.
Just about everyone involved with HCM has heard the name Dr. Barry J. Maron. Dr. Maron has devoted his entire career to hypertrophic cardiomyopathy and has been perhaps the physician most associated with HCM, having gotten his start in the 1970s at the National Heart Institute.
In a brand new autobiographical essay by Dr. Maron, he retells his recent experience being diagnosed and treated for heart failure.
When Dr. Maron experienced edema, difficulty lying flat, and trouble breathing, he rationalized it all away until he experienced a terrifying and life threatening episode, and even then, he decided to call an Uber to take him to the hospital instead of an ambulance!
Once hospitalized, doctors were able to get to the root of his problem and treat Dr. Maron appropriately. Having received the proper care, he is now feeling much better.
Dr. Maron’s article concludes with three main takeaways:
- Physicians should not diagnose themselves;
- Heart failure is treatable; and
- Listen to your cardiologist and live a healthy lifestyle!
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.
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.
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.
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.
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!
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.
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.