Apical Aneurysms in HCM

A recent paper published by doctors at Canada’s Peter Munk Cardiac Center looked at the implications of left ventricular apical aneurysms in hypertrophic cardiomyopathy,  This paper recommends that patients with an apical aneurysm of at least 2 centimeters consider prophylactic anticoagulant therapy to prevent stroke, as well as considering surgical placement of an implantable defibrillator to protect against sudden cardiac death.

A companion editorial suggests caution in basing aggressive measures on an aneurysm alone and says that all risk factors be considered together.

In 2017, HCMBeat covered a retrospective study of patients treated at Minneapolis Heart Institute and Tufts which discussed the risks for patients with apical aneurysms.  That can be found here.

Promising Data about Aficamten Presented at Meetings

Encouraging data about the Cytokinetics investigational drug aficamten was presented over the weekend at 2 scientific meetings held in Washington D.C.

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Myectomy: Still the Gold Standard for HOCM

An expert panel comprised of many of the world’s top HCM experts recently published a retrospective analysis in The American Journal of Cardiology which looked at septal myectomy over the last 60 years of practice.

 Beginning with the introduction of the procedure at the National Institute of Health in the early 1960s, this paper surveys the history of the procedure until the present day.  The conclusion of the paper is that myectomy remains the best treatment for patients with obstructive hypertrophic cardiomyopathy.

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A Professional HCM Society is Born

Social media erupted this week with exciting news for the HCM community  A new professional society specifically devoted to the study and treatment of hypertrophic cardiomyopathy has been launched. The name is the HCM Medical Society and its new website can be found here.

Founding members of the Board of Directors include many of the top HCM clinicians and researchers from around the world.

The HCM Society will focus on “clinical excellence, research and education” and will hold its inaugural meeting on Friday, September 30 in Washington D.C.

This is wonderful news for HCM patients, and HCMBeat hopes to have more about the future plans for HCM Society in the coming weeks.

Recommendations for HCM Depart from 2020 ACC/AHA Guidelines

A recent article by several noted HCM experts shares their recommendations for the screening and evaluation of patients with HCM using several scenarios commonly encountered in clinical practice. Notably, this paper makes several recommendations which differ from the 2020 ACC/AHA Guidelines for the Diagnosis and Treatment of Patients with Hypertrophic Cardiomyopathy (2020 Guidelines).

In particular, the article recommends:

  • Cardiac MRI with contrast upon initial evaluation, and every 3 – 5 years thereafter.  (The 2020 Guidelines recommend Cardiac MRI in certain situations, but not for all patients)
  • Genetic testing only in cases where a pathogenic or likely pathogenic mutation has been identified in a family member and in limited other circumstances (the 2020 Guidelines recommends genetic testing for all as part of the initial assessment)
  • Screening of family members 12 or older only (the 2020 Guidelines recommend screening of children of any age when genetic HCM is diagnosed in a family member and no later than puberty, in any event)
  • That HCM patients be disqualified from competitive sports. (The 2020 guidelines instead recommend a shared decision making approach).

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Aficamten Updates from Cytokinetics

At two recent meetings, South San Francisco biotech company Cytokinetics presented encouraging data on its experimental drug for hypertrophic cardiomyopathy, aficamten (formerly known as CYK-274). 

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Increased COVID Risk for HCM Patients

A recent retrospective study of HCM patients with COVID-19 concluded that HCM patients are at increased risk of hospitalization and death from a COVID-19 infection. 

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FDA Approves Mavacamten under Brand Name Camzyos

At long last, there is a FDA approved drug specifically intended for the treatment of hypertrophic cardiomyopathy.  After several years of testing, and based on the results of the groundbreaking EXPLORER-HCM trial, Bristol Myers Squibb’s new drug mavacamten, being marketed under the brand name Camzyos, is now available to HCM patients.

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Sports and HCM – Moving Toward Shared Decision Making

While competitive sports used to be frowned upon in the HCM literature, there is now some evidence that a patient’s risk from exercise is low when they have been implanted with an implantable cardioverter defibrillator (ICD). This feature from Medpage Today gives an overview of contemporary thinking about exercise in HCM patients.

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Traditional Cardiovascular Risk Factors Worsen HCM

According to this recent article in the European Heart Journal, traditional cardiovascular risk factors such as obesity, hypertension and diabetes are associated with more severe forms of HCM. Therefore, the authors suggest that these additional risk factors should be aggressively managed so as to limit their impact on HCM.