Category: Uncategorized

2020 AHA/ACC HCM Diagnosis & Treatment Guidelines Released – Updated With New Links

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A new article by Dr. Steve Ommen and Dr. Chris Semsarian entitled “Hypertrophic cardiomyopathy: a practical approach to guideline directed management” was published today in The Lancet. This blog entry has been updated to link to that article.

HCM Beat

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.

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Different Treatment for Non-Genetic Hypertrophic Cardiomyopathy?

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Many HCM patients, perhaps even the majority, are currently unable to identify the specific gene behind their HCM through genetic testing.  Despite this obvious difference, family screening, risk stratification and treatment standards are no different for patients who carry a HCM gene and those who do not have identified gene(s).

A recent article published in Circulation suggests that there ARE differences which should result in different treatment for this subset of patients.

In particular, non-sarcomere positive patients:

  • have a better prognosis, with lower rates of heart failure, sudden death, atrial fibrillation and stroke
  • Have lower incidence of family members affected by the disease
  • Are more likely to have additional medical conditions such as obesity, hypertension and diabetes

The article by Dr. Hugh Watkins, a British HCM and genetic expert, suggests that:

  • the risk to first degree relatives of this type of HCM patient is less than 50% and therefore, there is less need for repeated screening of relatives
  • Hypertension should be treated more aggressively in these patients.

Read more about non-genetic HCM here and more about screening these patients here on HCMBeat.

Patients Fare Best at HCM Specialty Centers

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A recent retrospective study conducted by doctors at Yale -New Haven Health System found that patients with hypertrophic cardiomyopathy fare best when treated at a specialty center using a team approach to HCM. The study found that this was especially true for patients coming from disadvantaged backgrounds who often fare worse outside of a HCM specialty setting.

The findings of this study suggest that patients with HCM are best served when referred to HCM specialty care instead of receiving care solely from general cardiologists.

2 Companies Testing Drugs for HCM

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Updated with recent data on mavacamten from ESC Paris, 2019.

HCM Beat

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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MyoKardia Announces Positive Result for Mavacamten for Treatment of HOCM

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This week, MyoKardia announced positive data on its experimental drug for HCM, mavacamten (formerly known as MYK-461), for obstructive HCM.

Continue reading “MyoKardia Announces Positive Result for Mavacamten for Treatment of HOCM” →

Broad Survey of HCM by Dr. Barry J. Maron

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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.”

 

New Educational Resource for HCM Patients

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As of July, 2018 the HCMCare.com website and app have been updated to include even more information for HCM patients. Check it out.

HCM Beat

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.

Click here to find on iTunes and Google Play.
HCMCare features video clips from the following physicians:

  • 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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CNN Chief Jeff Zucker Set for HCM Surgery

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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:

Wall Street Journal

Atlanta Journal Constitution

Los Angeles Times

Deadline

Variety

Hollywood Reporter

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

Chapter 4: MRI Safety for ICD & Pacemaker Patients

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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.

This study confirms previous findings relating to the safety of MRIs in ICD and pacemaker patients reported by HCMBeat here and here.

You can also read my personal experience trying to get a brain MRI in Los Angeles last year here.

Atrial Fibrillation: Treatable HCM Complication

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Updated to include editorial by Dr. Paolo Spirito in recent issue of Circulation.

HCM Beat

A recent paper by doctors at Tufts University’s HCM Center found that transient episodes of atrial fibrillation (AF) are treatable and do not often progress to permanent AF.

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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