A Risk Calculator for Sudden Death -Results of HCM-EVIDENCE Study

The HCM Risk–SCD Score

In 2014, the European Society of Cardiology (ESC) introduced a numerical predictor (the “HCM Risk–SCD score”) to assist physicians in identifying those patients at highest risk for sudden cardiac death who would benefit from the implantation of a prophylactic implantable cardioverter-defibrillator.

Using an algorithm generated by the answers to a series of questions, the tool estimates the 5-year risk of sudden cardiac arrest for any particular patient.  You can find the tool online here.

About the HCM-EVIDENCE Study

The reliability of this tool was recently evaluated by the HCM-EVIDENCE study, the results of which were presented at the recent ESC meeting in Barcelona.  This study examined the reliability of the HCM Risk–SCD score in 3,703 patients. The study looked at whether the scores accurately predicted the risk of sudden cardiac arrest (SCA) in particular patients.  The results of the study showed that the tool was useful in distinguishing high from low- risk patients.

Patients who were classified as low risk (whose score predicted less than a 4% chance of a SCA over a 5-year period) had an actual incidence of SCA of 1.4%, while those classified as high risk (with a score predicting a chance of SCA that was greater than or equal to 6% over a 5-year period) had a SCA incidence of 8.9%.

According to the British investigator who led the study, Dr. Constantinos O’Mahoney, “…for every 13 high-risk patients who receive an ICD as recommended by ESC guidelines, one patient could potentially be saved from SCD ...The study also showed that a low score on the HCM Risk-SCD calculator helped avoid unnecessary ICD implants in low risk patients, supporting the 2014 ESC recommendation not to implant ICDs in these individuals.”

Potential Impact of HCM-EVIDENCE Study

Dr. O’Mahoney added that while there is no way to predict and prevent all SCAs, the HCM Risk-SCD calculator help patients to better conceptualize the level of risk which can assist the shared decision-making process .

Dr. Nancy Sweitzer of the Sarver Heart Center at the University of Arizona was interviewed by MedPage Today about the study while at ESC.  Dr. Sweitzer observed that the HCM Risk-SCD calculator could assist in convincing a U.S. health insurer that an ICD is justified in specific cases, since the tool puts the risk of sudden cardiac arrest into quantifiable terms which have now been shown to correlate to actual outcomes.

[Editor’s Note – I tried the calculator out myself.  It suggested that I get an ICD.  I came to the same decision with the advice of several physicians 15 years ago. Though it has never been called upon to do its job, I am glad to have it, just in case.]

Guest Blogger – When a Seizure is not a Seizure – by Wendy Borsari

It’s strange to think that a chaotic arrhythmia in the heart might actually appear to be a seizure caused by something that has gone haywire in the brain, but with sudden cardiac arrest (SCA) this can sometimes happen.

This is the true story of what happened to my daughter.

On April 11, 2017, less than a week after her 14th birthday, my daughter suffered multiple cardiac arrests.  Two of these incidents were thought to be seizures by those who witnessed them.  And it wasn’t only her dance teacher who saw the first event who called it a seizure, but it was also the paramedic who arrived on the scene in time to see the second one.

After her muscles relaxed and she was still, the paramedic informed me that “it appeared as though” my daughter, “could have an undiagnosed seizure disorder.”  He relayed this information even after I had told him that she had Hypertrophic Cardiomyopathy and had had a previous sudden cardiac arrest that presented the same way.

As I listened to him, my mind was reeling.  My daughter had HCM, but could she also have epilepsy? What would this mean for the rest of her life?

Once hooked up to an EKG machine, it became apparent that the cause of my daughter’s events was cardiac; not neurological, but that didn’t make the situation any less horrifying for me or my husband.  While we’ve known for many years that our daughter was at risk of SCA, I had always imagined it would simply look like she fainted, without movement and breathing.

An ambulance rushed us to the local hospital where my daughter proceeded to have three more events, two of which caused her heart to stop completely!

The last time it happened, it took 2 full minutes of CPR to bring her back.

We were then told she needed to be med-flighted to Boston Children’s Hospital but they wouldn’t transport her until they took appropriate precautions in order to prevent another episode while in the air.

So, with a simple shot of local anesthetic to her neck, the on-call cardiac surgeon put a line into her jugular vein and down into her heart that would regulate her heart rhythm and keep her safe.

It was close to midnight by the time we arrived on the rooftop of Boston Children’s Hospital.  The pacer wire which had been placed in her heart worked, and she didn’t have any more events.  For the first time, I felt that she was safe and would be okay.

Two days later she had a dual chamber implantable defibrillator and pacemaker implanted.  We were all eager to go home and put the terrible events of the previous days behind us.

Though I’m still haunted by the events of that night, I’m so thankful that she was able to get the help she needed right away. But what happens to others whose cardiac events go unrecognized, or to the athlete who collapses on the field and looks like he or she is having a seizure?

I am sharing our story with the hope that it might bring awareness and possibly even save a life.

What Does Cardiac Arrest Look Like?

A person who is having a SCA will often become rigid with muscles seizing, as though suddenly made of stone. With SCA, breathing is non-existent.  You will sometimes hear a sound that is something like a cross between a loud snore and a gasp. This is called agonal breathing, which is actually not breathing at all. Agonal breathing is the brain’s attempt to override the body as it’s being starved for oxygen.  Agonal breathing is present roughly 40% of the time in a cardiac arrest, occurring during the short window of time that an AED shock would still be effective.

With a seizure, there will probably still be breathing, even though it may come in gasps.

It terrifies me to think that a bystander who witnesses a collapse might not reach for the nearest automatic external defibrillator (AED) if the event looked the same as my daughter’s.  Instead, they might call 911 and stand helplessly by, waiting for the paramedics and assuming that there was nothing to be done.

The events of that terrifying night in April will be with me for the rest of my life. I am forever changed by what I saw, and by the sounds I heard coming from my daughter.

However, if sharing my story with others might possibly save someone else’s son or daughter, it might help those awful sounds to fade from my dreams.


For more on how to distinguish a SCA from a seizure, see this link.  Please note that this page contains graphic videos which may be disturbing.

About Wendy

Wendy was diagnosed with Hypertrophic Cardiomyopathy at the age of 24. She comes from a family with a long history of HCM and sudden cardiac arrest. There have been 8 heart transplants within her immediate and extended family.

Wendy has 2 children, both of whom have been diagnosed with HCM. Wendy’s daughter was diagnosed at birth and her son at the age of 15.

Wendy now works with the Sarcomeric Human Cardiomyopathy Registry (SHaRe) and educates people about genetic cardiomyopathy through the website www.theshareregistry.org and through her “Affairs of the Heart” patient conferences held around the United States.

You can email Wendy at:  wborsari@shareregistry.org

Duchess Kate’s Sister, Pippa, Supports British HCM Charity

Pippa Middleton, who came to the public’s attention during the wedding of her sister Katherine to Prince William, has recently dedicated her efforts toward raising money for HCM genetic testing and research.  Middleton’s efforts are in honor of her late friend Miles Frost, who was lost to sudden cardiac arrest due to HCM in 2015. Frost’s father, British journalist and media personality David Frost, died from HCM just two years earlier in 2013, but this information was never communicated to the family.

The Miles Frost Fund, a partnership with the British Heart Foundation , helps families who have lost a member to a sudden death obtain genetic testing in order to learn if other family members are similarly affected. The Frost Fund also funds research by U.K.researchers working towards finding a cure for HCM.

Parent Heart Watch Featured on TODAY

The work of Parent Heart Watch – an organization formed by parents of children who died due to sudden cardiac arrest -was featured on the Today show this week.

Parent Heart Watch works tirelessly so that AEDs are available in schools and on playing fields around the U.S. to ensure that children will not fall victim to SCA.

Great work PHW!

Lindsay Davis: Using Her Big Heart to Help Others With HCM

This article, published in this week’s Women’s Health magazine, features the former ballerina and beauty queen turned vocal patient activist. These days, Lindsay has focused her efforts on saving lives from sudden cardiac arrest.  Lindsay’s efforts in the state of Ohio have resulted in proposed legislation to identify student athletes at risk of sudden cardiac arrest, while her partnership with the American Heart Association is steadily making CPR and AED training a graduation requirement in high schools across the nation.

Watch for more life-saving advocacy from Lindsay in the future.  She is clearly much more than another pretty face!

Updated to include a video of Lindsay discussing her implantation with a S-ICD device.

Exercise Does Not Trigger Most HCM Deaths

According to research presented at the 2016 European Society of Cardiology Congress today, sudden cardiac arrest from HCM, which has long been thought to result from exercise, is actually more likely to occur at rest, or even during sleep, according to Dr. Gherardo Finocchiaro, a cardiologist at St George’s University in London.  Dr. Finocchiaro also pointed out that of the 184 HCM patients in his study, almost 80% had no previous symptoms of HCM, and only 1 in 5 had been diagnosed with HCM before their deaths.  Interestingly, most of the sudden deaths from HCM analyzed in the study occurred in patients in their 30s and 40s.

New Model for Predicting SCA?

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Doctors at the University of Pennsylvania have created a new model which they say can predict the risk of sudden cardiac arrest in people without known heart disease.   According to the article, published recently in Circulation, low levels of albumin, a protein commonly tested in routine blood panels, is a novel risk factor among the twelve factors identified.  This article in Cardiac Rhythm News offers more details.