The expanding field of personalized medicine has not left hypertrophic cardiomyopathy behind. In fact, two companies are currently developing targeted gene therapies for HCM patients.
In a recent study, researchers examined whether cardiac MRI results might help predict which patients would would go on to develop atrial fibrillation (AFib) that was serious enough to require hospitalization, require electrical cardioversion or catheter ablation, or identify those patients who might go on to develop permanent AFib.
The study found that the major predictors of these serious AFib consequences in HCM were those who were of older age, those with an increased BMI (this was especially important in patients under age 33), increased left atrial volume index as seen on cardiac MRI (this was especially important in middle-aged patients), reduced left atrial contractile function (this was especially important in middle-age and older patients), and moderate or severe mitral valve regurgitation.
The researchers concluded that using cardiac MRI to measure left atrial volume and contractile function might help medical providers ability to intervene before major AFib develops in HCM patients, specifically by helping patients find ways to reduce their weight and by treating mitral regurgitation and left atrial function more aggressively.
HCM specialists at Tufts Medical Center and Toronto General Hospital have devised a formula which they hope will help predict which HCM patients may go on to develop atrial fibrillation (“AFib”) over time. This tool can assist doctors in determining which patients are at highest risk so that these patients can be closely monitored and treated appropriately. AFib can be extremely dangerous for HCM patients since it can precipitate a stroke if not appropriately treated.
Because existing tools to predict atrial fibrillation have not proven to be accurate for HCM patients, the researchers studied 1900 HCM patients with the goal of devising a new tool to help HCM patients and their physicians learn their personal risk for AFib over a 2 and 5 year period.
CALCULATE YOUR RISK SCORE:
The formula works as follows: Find your left atrial diameter on your most recent echocardiogram report. On the chart below, find the number of points (listed in the 2nd column) that corresponds with your left atrial measurement (measured in millimeters). Then, find the number in the 2nd column that corresponds to your current age range. Add these two numbers together. From that sum, subtract the number listed in the 2nd column that correlates with your age range when you were first diagnosed with HCM. Lastly, if you have heart failure symptoms, add in 3 points. The total is your score.
You can calculate using the table below, or you can use this online calculator which gives you your 2 and 5 year risk.
WHAT DOES YOUR RISK SCORE MEAN:
Low Risk scores are between 8 – 17
Intermediate Risk scores are between 18 – 21
High Risk scores are between 22 – 31
As always, discuss with your doctor. Everyone has individual factors which will influence their degree of risk. This tool is intended as a general guideline to facilitate discussion with your physician.
According to a recent retrospective study at Oregon Health & Sciences University, appropriately selected patients 65 or older who underwent septal myectomy for obstructive hypertrophic cardiomyopathy (HOCM) have surgical outcomes similar to younger patients. Therefore, older age should NOT be an automatic disqualifier for myectomy. All potential treatments for outflow tract obstruction should be considered, with age being only one of many factors influencing the decision.
I was recently asked by the good people at MyoKardia, now a subsidiary of Bristol Myers Squibb, if I would author a plain language summary of an article to be published in the journal Future Cardiology. This article summarizes the results of the EXPLORER-HCM trial, which showed that mavacamten, a new type of drug known as a “myosin modulator,” was successful in reducing HCM symptoms in subset of patients who took the drug during the clinical trial.
I was so fortunate to have Dr. Anjali Owens of the University of Pennsylvania Center for Inherited Cardiac Disease join me as co-author on the article. I also want to thank the teams at Bristol Myers Squibb and Cello Health Communications/SciFluent who supported us on this project. And, I so grateful to the former MyoKardia team and the other members of the EXPLORER-HCM Steering Committee who gave me the opportunity to witness a groundbreaking clinical trial from the front row.
This is the article. When you access the page, click to view the very informative short video, located in the upper right corner of the page, which explains how the drug works in very brief and concise terms.
Check it out!
Cynthia Burstein Waldman, Founder and Editor, HCMBeat
More positive data about the Bristol Myers Squibb experimental drug mavacamten was revealed at last weekend’s American College of Cardiology meeting and simultaneously published in The Lancet.
The data showed improvement in how patients felt taking the drug, as reported and quantified by the patients themselves. The clinical trial participants filled out a questionnaire called the KCCQ, or Kansas City Cardiomyopathy Questionnaire, 6 different times over the 38 weeks that the trial was ongoing.
Analysis of these responses showed that just over a third of patients who took mavacamten reported a substantial improvement in symptoms, though not all participants responded to the drug. And, it is important to note that the improvements seen by patients returned to baseline once mavacamten was discontinued.
Mavacamten, formerly known as MYK-461, was developed by the San Francisco based company MyoKardia and acquired by Bristol Myers Squibb late last year for $13.1 billion.
You can read more about these results in
and here is an interview with Dr. Jay Edelberg of Bristol Myers Squibb about mavacamten.
DISCLOSURES: 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 EXPLORER STUDY. until recently CYNTHIA also SERVED ON MYOKARDIA’S PATIENT ADVISORY BOARD.
Today, HCMBeat.com surpassed 100,000 views!
Big thanks to everyone who has supported HCMBeat over the last 5 years, especially all of the doctors and researchers who spend their days working to improve the lives of patients with HCM.
I hope that this blog will continue to inform the HCM community about the latest news and information related to hypertrophic cardiomyopathy .
The last few years have been exciting for HCM patients and the future looks even brighter!
Happy Mother’s Day to all.
Cynthia Burstein Waldman, HCM Patient, Founder and Editor, HCMBeat.com
A recent Chinese study found that patients with hypertrophic cardiomyopathy who also suffered from clinical depression had a higher risk of sudden cardiac death and/or heart failure.
The researchers behind this study hypothesize that psychological treatment for depression could improve clinical outcomes in these patients and they suggest that further research is warranted.
The FDA has announced a Class I recall of certain models of Medtronic implantable defibrillators and cardiac resynchronization therapy defibrillators. The statement from the FDA can be found here.
The recall impacts Medtronic’s Evera and Visia brands of ICDs as well as its Viva, Brava, Claria, Amplia and Compia brands of cardiac resynchronization therapy defibrillators (CRT-Ds) implanted from August 31, 2012 to May 9, 2018. The problem is described as “an unexpected and rapid decrease in battery life” caused by a short circuit. The defect may cause devices to go from recommended replacement time (when your alarm sounds to notify you that it is time to schedule a replacement surgery) to full battery depletion in just one day. Normally, you would have approximately 3 months from the time that the alarm first sounds to schedule a procedure to implant a replacement.
According to this article, there have been 444 complaints and 264 medical device reports about this issue which impacts 239,171 devices.
To look up your device by product name, model or serial number to see whether it is impacted, click here.
The box in the upper right corner labeled “Advisories For This Model” will tell you if there are any advisories for your device. If this particular recall affects your device, you will see the same advisory which is circled in red in the photo below.
Patients are advised to continue routine follow ups and use the Care Link Monitoring System.
And, as always, you can call Medtronic Patient Services with any questions at: (800) 551-5544
(M – F, 8am – 5pm Central).
A recent study published in the European Journal of Preventative Cardiology found that a 12-lead electrocardiogram (EKG) was not useful as a screening tool to determine which children were at increased risk of sudden death and therefore, a candidate to receive an implantable defibrillator.
The full article can be found here.
A recent study published in the European Heart Journal using data collected from the SHaRe Registry found that while childhood onset HCM (15% of all HCM) is far less common than adult onset HCM, it is often more serious.
Children with HCM are more likely to have sarcomere mutations, have a higher risk of ventricular arrhythmias, and are twice as likely as adults to require advanced interventions like ventricular assist devices, heart transplant or other advanced heart failure therapies.