A recent study looked at what researchers claim is the largest cohort of HCM patients ever studied. This study found that HCM patients are at substantially elevated risk for atrial fibrillation and heart failure, and have significantly higher mortality rates than that of the general U.S. population. Therefore, the researchers conclude, there is much more work needed in order to solve the riddle of HCM.
A recent study by doctors at the Cleveland Clinic found that current guidelines used to assess risk of sudden cardiac death (SCD) in HCM fall short when applied to the population of patients with the obstructive form of HCM (HOCM).
The study looked at both the European Society of Cardiology (ESC) and American College of Cardiology (ACC)/American Heart Association (AHA) guidelines, and found that both sets of guidelines came up short in predicting SCD. In particular, the study found that patients who had previously undergone myectomy had a reduced risk of SCD that is not accounted for in existing risk models.
Conversely, the study found that patients with atrial fibrillation had a higher risk of SCD, which is also not reflected in the existing risk models.
A companion editorial by Dr. Harzell Schaff of the Mayo Clinic explains the likely reasons for the myectomy findings, while a second accompanying editorial by Dr. John Jefferies of Cincinnatti Children’s Hospital (who has recently accepted an appointment at the U. of Tennessee Health Science Center in Memphis) maintains that the ESC and ACC/AHA guidelines should be changed to reflect the lower SCD risk following myectomy.
As of July, 2018 the HCMCare.com website and app have been updated to include even more information for HCM patients. Check it out.
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.
- 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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According to this study published recently in the Journal of the American College of Cardiology, whole genome testing may sometimes be used to identify the gene(s) responsible for HCM when targeted genetic testing (the type used in the clinical setting) has been inconclusive.
In particular, the study found the responsible gene(s) in 9 of 26 families (20%) in whom targeted testing had previously been inconclusive.
When used as the initial form of genetic testing, whole genome sequencing identified the responsible HCM gene in 5 of 12 families, or 42%.
According to this article in Wired U.K., a whole genome sequencing test costs about $600 and takes just a few weeks to complete. On the other had, the cost of data storage necessary to store such a large amount of collective data is, according to this article, prohibitively high.
If not for everyone, perhaps whole genome sequencing could be used in families where traditional genetic testing has proven inconclusive. Time will tell.
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:
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
This article in Cardiovascular Business discusses the financial benefit to a hospital that adds a center for the treatment of HCM. In particular, hospitals can expect to see higher volumes in the areas of echocardiograms, cardiac MRI, and electrophysiology.
This recent article published in Nature discusses several real-life scenarios in which patients were mistakenly diagnosed with serious genetic cardiac conditions, including HCM, as a result of erroneous direct-to-consumer genetic testing.
These misdiagnoses directly resulted from misinterpretation of raw data by third party interpretation services that were working with raw data provided to them by direct-to-consumer genetic testing companies.
After medical testing, none of the patients discussed in the highlighted cases were ultimately found to have disease or be in need of medical intervention, though all underwent unnecessary medical testing and/or invasive procedures. Some even made radical lifestyle changes as a result of the erroneous genetic information.
This article demonstrates the unreliability of direct-to-consumer genetic testing, which has the potential to cause great upheaval to both patients and the medical system.
As always, patients seeking genetic testing should do their homework. Genetic testing for heart conditions is best when done by the experts – cardiac genetic counselors!
Editor’s Note: Doug Rachac found out that he himself needed an ICD while employed by device manufacturer Medtronic. He now uses his personal experiences to help other device patients learn how to live happily with their implantable devices.
When cardiac patients receive a pacemaker or defibrillator, they often have questions about their new “friend.” They wonder if this new device has limitations, and what those limitations might be.
And they may find that the internet can be, at the same time, both helpful and misleading. Accurate information is out there, but separating truth from fiction is the hard part. To learn the truth, patients turn to their doctors, nurses and clinic device techs for answers, not realizing that sometimes these professionals also give advice based on outdated or incorrect information.
When a patient wants to know if it is safe to scuba dive with their heart condition, they should ask their physician. However, if they want to know how deep it is safe for them to dive with their specific implanted device, instead of asking their physician, they should instead direct their question to the engineers of the device manufacturer who designed and tested the device.
Patients should speak with their doctor to learn if their health condition limits them from certain physical activities (such as scuba diving), but questions about the limitations or capabilities of devices and leads should usually first be directed to the manufacturer of the device – the best source of up-to-date, accurate information. Whenever I am looking for up to date, accurate information about my device, I start with Medtronic, the manufacturer of my implanted device.
Patients can easily find the make and model of their device, as well as learn how to contact their device manufacturer, by calling the Patient Services number located on their Patient ID card.
In this blog article, I’d like to share with you some information regarding two of the most common misconceptions relating to implantable devices out there: 1) magnets are dangerous to implanted devices like pacemakers and defibrillators; and 2) it is unsafe to walk through airport/venue security.
All of the information I discuss below can be found, if you dig deep enough, on each specific manufacturer’s websites.
Magnets: Should We Be Afraid?
All companies advise patients to keep magnets at least 6-8 inches away from their devices for their safety. But the simple answer to the question is NO! You do not need to be afraid of magnets. However, you do need to be aware of how a magnet interacts with your device when you come into contact with one.
A magnet will NOT:
- Scramble the device programming
- Turn the device off
- Turn off pacing
- Damage the device in any way
These common misconceptions are based on devices that are over 10 years old, or are based on a misunderstanding of the truth. In most industry devices a magnet will interact with a pacemaker and defibrillator in the following ways:
Pacemaker or Cardiac Resynchronization Therapy Pacemaker (CRT-P):
A magnet placed on or near a pacemaker/CRT-P device will force the device to pace at a constant, preset rate. Different companies have different preset rates. Medtronic is set to pace 100% of the time at 85 beats per minute (BPM). When the magnet is moved away from the device, the device will immediately revert back to the programming that was input by the doctor or device tech at implant, or that was modified during a subsequent interrogation session.
Defibrillator or Cardiac Resynchronization Therapy Defibrillator (CRT-D):
In most industry devices, a magnet will “inhibit detection” which is a fancy way of saying the device won’t shock you if needed. Once the magnet is removed, the shock function reverts back to normal.
This function is a normal design feature of the devices designed for use by clinicians and doctors. In both types of devices, a magnet will in no way harm the device or alter the programming. The effect of the magnet is only temporary. Once the magnet is moved 6-8 inches away from the device, the device’s normal programming will resume. Some everyday items such as iPad covers, purses, and children’s toys may contain magnets which are strong enough to trigger the magnet response in our devices. However, simply moving those items 6-8 inches away from your device will resolve the issue. Remember, even if you do encounter a strong magnet or magnetic field, it will not harm your device in any way. Simply move away from the magnet/magnetic field and your device will return to its normal operations.
Let me reiterate: a magnet will in no way harm your device, shut it off, or alter the programming.
There was a time when walking through airport or venue security with an implanted device was considered dangerous. This was back when our devices included an electrical component called a Reed Switch. But, over a decade ago, devices evolved after safety issues forced industry to eliminate the Reed Switch in all implanted devices. At the same time, a new international standard emerged setting limits for how much magnetic energy an implanted device needed to be able to withstand. The level is high enough that most forms of magnetic energy encountered in our daily lives are insufficient to interact with our devices in any meaningful way. There are a few exceptions, such as the electromagnetic energy generated by a hydroelectric dam. But, airport archways and hand wands do not generate that level of energy. Companies like Medtronic simply suggest that a patient walk through the airport archway at a normal pace, and that wands are not waved repeatedly over the device multiple times. Aside from those precautions, patients do not need to avoid airport or venue security in any way. Millimeter wave scanners (the one that rotates around you) are also safe to use.
Let me say it again: Pacemaker/ICD/CRT patients do not need to fear airport/venue security checkpoints. Simply walk through the archway at a normal pace, and ask that wands not be waved back and forth over the device repeatedly.
Living with our devices can be stressful enough. It’s my belief that having accurate information is the first step in being able to live the life we want to live, not the life we think we are now forced to live.
My physician was clear when he said, “Your device is there to protect you and allow you to live your life. It is not there to restrict you in any way.”
It’s time to dispel the information of old, and to start living our lives without these old fears. Our devices have evolved over the years so we need not fear magnets or security lines any more. If you would like to learn more about me and living with an implantable device, I have uploaded several videos to my YouTube Channel. You can find specifically about devices, magnetic fields and airport security here.
About Doug Rachac:
Doug Rachac received his implanted Medtronic defibrillator in 2014 due to several episodes of syncope and documented non-sustained ventricular tachycardia. He spent 14 years working for Medtronic, where he worked in multiple areas, including Customer Service, Education, and Quality. He left Medtronic in 2015 to recover, volunteer and to be the world’s best stay-at-home dad. He now advocates for device patients and consults with the medical device industry on patient engagement and patient focused initiatives. You can find him on Twitter @HankEPants.
A high-voltage song from the band AC DC rocks my headset, jolts my brain, legs, arms, and attitude. Deep below the bone-crunching guitar of “It’s A Long Way to the Top (If You Wanna Rock ‘N’ Roll)”, an accompanying hard-driving riff powers something bigger; it goes thump, thump, thump, thump, thump. I’m winded, gasp for air, and my face turns red. When I press my fingers against my sweaty neck, I count 125 thumps a minute and relish every breath.
I’m running sprints and have only been able to listen to my heart beat like this since open-heart surgery at Mayo Clinic. I was diagnosed with hypertrophic cardiomyopathy (my story) a few years ago, a condition that inhibits blood flow because the heart muscle is too thick. I tried medicine, but decided that a procedure to remove a part of that muscle, a septal myectomy, was my best option. Immediately after surgery, a new sound with a steady rhythm and powerful cadence played through my body; this music to my ears was my new and improved heartbeat, which continues today, nearly three years later.
Over this time, I have seen a lot of similar questions about HCM surgery on social media sites. After my positive experience, I wanted to share some HCM CARE tips to help patients and families prepare for surgery:
Help – Prior to surgery; get help at home and at work. Line up a caregiver, friend, or family member for two weeks after surgery. My wife was amazing and without her, I believe I would have suffered setbacks. If you work and have a leave of absence policy, make arrangements as early as possible and try to detach from work during your recovery. In my case, that helped me recover physically and mentally.
Clean – Follow wound prep instructions precisely before and after surgery. Prepare as many laundered clean towels and wash cloths as you can before leaving for the hospital, if possible, at least 20. These need to be clean every day when you get home, so load up. In my case it was also recommended to use liquid, not bar soap.
Meals – Try to buy or prepare two weeks of frozen dinners and lunches. You might not feel like bending over after you return home. This also offers the opportunity eat healthy.
Chair – If you have a seat to recline in, great. I didn’t, and that would be one of my top recommendations. If you can afford to buy a chair or rent one of those seats that props you up, please consider that. The first few weeks after surgery, I felt like a turtle trying to roll upright after tipping over.
Area restaurants and hotels – I live in Rochester, Minnesota and work at Mayo Clinic in Communications, so this experience was easier for me, but a lot of people ask for a list of hotels and restaurants.
Rehab – Studies show that people who participate in rehab have an improved long-term outcome. When I came home, my wife would take me to the mall and I would shuffle from store to store until I built up enough stamina to work out. This report from our Mayo Clinic News Network on my cardiac rehab from about two years ago shows the benefits. Also, if it is offered to you, I recommend massages by an occupational therapist who can work the muscles that have been traumatized. I began this in the hospital and believe it helped me.
Exhale and inhale – My biggest surprise was low lung capacity. Even though my daughters tried to make me laugh in the hospital, I could barely make a squeaking sound. I used a spirometer (not quite like the one in the picture but a smaller version) to learn how to exhale and inhale after being placed on the heart-lung machine during surgery. The goal or incentive of the spirometer is to open the air sacs in your lungs, making it easier to breathe deeply and keep your lungs clear. It is believed that proper usage may speed up your recovery time while reducing your risk of developing pneumonia or other breathing problems.
Additionally, no matter how many pictures and videos as you see before your surgery, it’s still surprising to see wires and tubes coming out of your chest.
Everyone’s experience is unique, so always please keep that in mind. I would go through it again in a heartbeat (pun intended) and as I have said often, the team at Mayo exceeded all of my expectations.
The intense pain following surgery subsided after the first few days and today, I feel decades younger. I have high exercise tolerance and on most days, my overall energy level is very good. However, I occasionally experience bouts of low energy, and when that happens, I try to lay low and sleep. Atypically, my appetite is less than it was prior to surgery and my food tastes have changed. That’s good in that I lost a healthy amount of weight, but bad because I don’t enjoy my wife’s fantastic cooking like I used to. While my heart is doing well, I have experienced frequent back pain over the past few years. I have gone through physical therapy and am told my core is strong, but I continue to address this challenge.
Social media was an invaluable resource to learn about HCM and preparing to make a decision about surgery. In addition to Mayo’s online information about HCM surgery I also found support on the Hypertrophic Cardiomyopathy Forum on Mayo Clinic Connect as well as on Facebook in the Mayo Clinic HCM Patients Group and on the blog by fellow patient Cynthia Burstein Waldman, HCMBeat.
I felt prepared because I read posts and blogs from other patients, conducted a lot of research, and felt informed going into this. I do wish that I would have connected with more patients to learn about the post-operative challenges after going home. My surgery was a great success and I hope this post can help make your experience even better.
For those of you who have had surgery, what tips would you add ?
About Ron Petrovich:
Ron Petrovich has had a long career in journalism and public relations and has worked at Mayo Clinic since 2010. He currently serves as Communications Director, News and News Delivery in Mayo Clinic’s Department of Public Affairs.
Ron was diagnosed with HCM while an employee at Mayo Clinic (see his story here) and now generously shares the benefit of his experience in order to help others on the same journey. You can find him on Twitter @RonaldPetrovich.
Editor’s Note: If you are thinking of visiting Mayo Clinic for HCM treatment, you may also find this post from the SADS Channel blog informative.
According to a recent study by doctors in the Netherlands published in the journal Circulation: Heart Failure, women who had undergone septal myectomy had more diastolic dysfunction and myocardial fibrosis than men who had also undergone myectomy.
Hence, the researchers suggest that sex-specific treatment for HCM may become customary and should be a subject for future inquiry.
These findings raise concern, especially when looked at in conjunction with a recent study by doctors at the Mayo Clinic who found that women with hypertrophic cardiomyopathy have a statistically reduced rate of survival when compared to men with HCM.
Here’s hoping that upcoming researchers will focus their efforts on improved outcomes for women with HCM.
According to this press release, MyoKardia expects to dose the first patient in the EXPLORER-HCM trial of mavacamten (formerly known as MYK-461) for obstructive HCM in the second quarter of 2018.
MyoKardia says that it expects 220 patients to enroll in the 30 week long trial. These patients will be randomly assigned to receive either mavacamten or a placebo. Participants will also be able to continue on their normal beta blockers or calcium channel blockers.