LGE Seen on MRI Top Risk Factor for HCM

To date, risk assessment in patients with hypertrophic cardiomyopathy (HCM) has largely focused on whether there is a need for an implantable defibrillator by evaluating an individual patient’s short term risk of sudden death, but has not looked at the long term risks of heart failure and other adverse outcomes over time.

A recent retrospective study by European HCM specialists asked the question of whether the addition of advanced imaging could assist in pinpointing patients at high risk of heart failure and/or other serious HCM complications over time. 

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HCM Genes Common in General Population

A recent study sought to find how common genetic cardiomyopathies are in the general population. 

By studying United Kingdom Biobank samples from 200,643 people who had undergone complete genetic sequencing, researchers established that genes linked to HCM are found in one out of every 149 people! 

More than half of those identified as having a HCM gene had a mutation in the MYBPC3 gene, making it the most common HCM gene in this sampling.

Jardiance for HCM Patients?

A recent study published in the American Journal of Cardiology found that patients with non-obstructive hypertrophic cardiomyopathy and diabetes showed significant improvements to diastolic heart function and BNP (a hormone produced by the body which indicates heart wall stress) from the use of a drug used for diabetes known as a sodium glucose co-transponder 2 inhibitor (SGLT2).  

This class of drugs is commonly used to lower blood sugar in diabetics.  One SGLT2 in common usage is Jardiance (empagliflozin).

Since treatments for non-obstructive HCM patients are so limited, this class of drugs presents a potential treatment opportunity for non-diabetic HCM patients as well. 

Hey all you researchers out there – sounds like a good clinical trial!

HCM Impact on Mental Health

A recent study by Korean researchers found that HCM is associated with mental health disorders, especially during the first year after a HCM diagnosis.  

This study only scratches the surface of the topic, but it does bring up an important point which we hope that medical providers will take to heart:  the mental health of HCM patients is just as important as their physical health and sometimes, mental health referrals and/or medications may be a necessary part of the HCM treatment plan.

Mavacamten Approved in Canada

Bristol Myers Squibb’s first in class drug  mavacamten (brand name Camzyos) has been approved by Health Canada for the treatment of obstructive hypertrophic cardiomyopathy.

The Future of HCM Treatment

Doctors from the University of Pennsylvania this week published an overview of current therapies available for the treatment of HCM.  While this article mentions all of the old standards – beta blockers, calcium channel blockers, myectomy and alcohol ablation, the real focus is on the future of HCM treatment. In particular, the article describes several new and/or experimental therapies which look promising for the future.

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Ranolazine Improves Chest Pain in HCM

A recent paper by Italian HCM specialists found that chest pain completely resolved for 73% of patients who took the drug ranolazine. The researchers also found that the drug was safe and well tolerated.  In addition, it reduced arrhythmias for those whose arrhythmias were triggered by ischemia and it improved biomarkers in HCM patients.

This study is a follow up to the 2018 RESTYLE-HCM study, which found that while ranolazine did not improve heart failure symptoms in HCM, it was useful for treating chest pain.

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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