Unfortunately, this study which was conducted by Dr. Perry Elliott and his colleagues at University College London, found that trimetatazidine did not improve exercise capacity in these patients. Following the results of this study, trimetazidine will now join ranolazine and spironolactone in the compost heap of drugs which tried and failed to improve HCM symptoms. While a third drug, perhexiline, was found to improve symptoms for non-obstructive HCM, its limitations, including potentially serious side effects, stand in the way of its common usage.
Though seemingly a more benign form of the disease, in fact, patients with non-obstructive HCM share a similar risk of sudden death and heart failure with their obstructed counterparts. Because treatments available to treat non-obstructive disease have been largely limited to beta blockers, calcium channel blockers and diuretics, there is a great need for new medications directed at this problem.
The problem with setting up HCM Drug trials is complicated by several factors:
Different Types of HCM: There appear to be multiple kinds of HCM. Some are genetic and some are not. We are now at a place where treatments for HCM should become more individualized.
Difficulty in Recruitment: It is difficult to recruit enough patients to take part in these trials. Patients often travel considerable distances for care at dedicated HCM Centers. Multi-center trials provide a potential way to get around this issue.
Duration of HCM: HCM is a disease process which takes place over decades which is not easily studied in a clinical trial of limited duration.
The Unknown: Timing of interventions may be critical, and it is hard to know the proper window of time to target. The bottom line is that there is still so much that is unknown, HCM researchers much continually adapt as knowledge of the disease process unfolds.
In conclusion, Dr. Day suggests that while medical researchers continue to test new drugs and push forward studies of HCM, physicians should counsel their patients that in addition to medication, lifestyle choices like proper nutrition and exercise will improve their clinical course and the overall outlook for HCM patients.
I had open heart surgery (a septal myectomy) to treat my hypertrophic cardiomyopathy in 2006. I went back to Mayo twice for the two years following the surgery, but after that I hadn’t felt the need to return since I was regularly following up with my local cardiologist. In April of 2018, it had been almost ten years since I had been back to Rochester. So, I decided it was time to take a trip and make sure that all was in order.
PREPARATIONS FOR MY VISIT
I called in January for an appointment, and was told that appointments are made only as far ahead as three months. Because I wanted to wait until the weather warmed up, I decided to wait to schedule until April when I was pretty sure that the snow would be gone. Sadly, that was not the case when I arrived. Nevertheless, this Southerner was not deterred by a little snow. And luckily, the skyways and subways provide easy access to the Clinic, regardless of weather.
A few weeks before I left I received a schedule in the mail of the tests I would be having on the first day of my visit.
Then, I made my plane reservations. I have learned that it is easier for me to fly into Minneapolis and take a shuttle from the airport than to fly into Rochester. It saves having to change planes, and the shuttle is easy and affordable.
ARRIVING AT THE CLINIC
I just beat the snow when I arrived on Sunday night. When I arrived in my hotel room, I was crestfallen to realize that I could not find my schedule which had me reporting to the lab for blood tests bright and early at 7:00 a.m. I wondered whether I would need to arrive even earlier than 7:00 a.m. in order to get another copy of my printed schedule.
Then I remembered that since my last visit, Mayo Clinic had added an online patient portal which seemed to have a lot of things in it, so I decided to check that before I completely panicked. Once I remembered my password, I was able to log into the website where I was asked if I wanted to download the Mayo Clinic App. I thought that seemed like a good idea, since I was there, wanted to get the benefit of the full experience, and my hotel had free wi fi, so why not? I downloaded the app, and then I was instantaneously able to see my full schedule along with all of the locations. It even provided me with maps of how to find them which came in handy since it had been awhile.
The knowledge that I was prepared for morning allowed me to go to sleep feeling that I had all under control.
BEATING THE SNOW
On this trip to Mayo Clinic, I stayed in the downtown area where I was able to walk through the skyway and subway to the Clinic. This turned out to be fortuitous, because even though I waited to make my appointment in April when, I was told, the snow would be over for the year, this year was a notable exception! A mid-April blizzard delivered more than a foot of snow.
My Minnesota friends, who usually mocked my Southerner’s fear of snow, admitted that I was right to be worried. I arrived just as snow flurries started falling from the sky. Fortunately, the snow held off as my airport shuttle traveled the highway from Minneapolis, but as soon as I checked into my hotel room, the flurries were visible from my hotel window. It snowed all night, and it was icy in the morning. Therefore, I was happy to be able to stay entirely inside, thanks to the Skyway and Subway system. (For those who are having surgery, note that it is also possible to stay across the street from St. Mary’s Hospital and take a shuttle bus when you need to visit the downtown clinic. )
I left my coat in the hotel room while I donned my tennis shoes for the trek to the Clinic. Note: Comfortable shoes are a must! There’s lots of walking involved in a Clinic visit (unless you have someone to push you around town – there are lots of wheelchairs available at the entrances just for the asking). I’d left my FitBit at home, but I think I must have logged about 13,000 steps that day.
So, unless you are visiting Rochester in May – September when your odds of snow are low, it is safest to stay in one of the many hotels which are attached to the Clinic. For this visit, I chose the Hilton Garden Inn which proved to be affordable and conveniently located.
My first appointment was scheduled for 7 am and I had been instructed to fast for the previous 12 hours, which even included limiting liquids. Because I had flown in the night before, it seemed I was dehydrated, which turned out to be problematic when they tried to take my blood. I almost fainted twice. My phlebotomist called in her supervisor after two glasses of water and a second attempt on the opposite arm produced the same effect. Ultimately, they got me an ice pack for my forehead which did keep me from fainting so on the third attempt they were finally able to get all the blood they needed. They started making slides while I drank an orange juice and ate a power bar, before moving on to my next test, a chest x-ray.
If you are worried about passing out from the blood draw the way I almost did, check with your doctor ahead of time to see if it’s OK to drink some water before your schedule starts. It might just save you from the same issue. From now on, I will be drinking water!
I was running a bit behind due to the unforeseen complications, so I was a little worried that I might have thrown off the scheduled times.
Not to worry, they were able to get me in when I arrived. The schedule at both the Chest X-ray and EKG stations is flexible: It doesn’t matter if you are early or late, their schedule allows for this.
The chest x-ray and EKG experiences were very similar to my previous visits. They get you in and out pretty quickly.
Whereas on past visits my echos were done at St. Mary’s Hospital, on this occasion my echo was performed in the Gonda Building at the Clinic. The young man who performed my test was a military vet who was finishing his last months at Mayo before going off to graduate school to learn how to assist with cardiopulmonary bypass surgery.
He was very thorough, spending about an hour taking images before he left the room to review the images with the supervising doctor. He left me to my own devices in the examining room for about 15 minutes so I used my phone to surf the internet. He came back and told me that all was well, so I was free to leave.
LUNCH AND SHOPPING
My next appointment with my cardiologist wasn’t until the afternoon, so I took an hour or two to do some shopping and eat lunch. The cafeteria is always pretty full so I decided to explore the subway tunnel which leads to the Kahler hotel. Some clothes caught my eye, so I tried on and bought a few blouses before continuing on to a nearby local lunch place. Lots of shopping can be found in the clinic area, including the University Square Mall which is an easy walk between appointments.
THE HCM CLINIC APPOINTMENT
After lunch, the results of all of my tests were available, so I was able to head to my consultation with my cardiologist, Dr. Steve Ommen, Director of the Hypertrophic Cardiomyopathy Clinic. Once they called me in, they took my blood pressure and I had a brief meeting with one of the nurses in the HCM Clinic. Then, Dr. Ommen came in and we discussed all of my test results and he made some recommendations for a medication change. After discussion, he decided to cancel the stress test which had been scheduled for the afternoon, since he said that the results would not impact his recommendations for my care. (Note: Though a V02 stress test has been scheduled for all four of my visits to Mayo, I have only ever actually undergone this test one time. Mayo intentionally schedules the test after you have met with the cardiologist so that s/he and you can decide if it is necessary or will provide useful information. Also, some patients who visit the HCM Clinic will be scheduled for a cardiac MRI. Since I already have an implantable defibrillator, this test is not ordered for me.)
That night in the hotel room, I noticed that my blood test and xray results were already up and online though the Mayo app. The only test results I was unable to access at that time were those from my echocardiogram. All test results, including my echo, were mailed to me within a few weeks of my appointment, and as of this writing, 4 months later, all results, including the echocardiogram, are reflected in my electronic record.
SIGHTSEEING AT MAYO CLINIC
Finished with all my appointments, I was able to spend the rest of my visit to Rochester sightseeing.
When at Mayo Clinic, it is imperative to spend time taking in the art and ambiance that makes a visit so special. On my first visit, my husband and I described Mayo as “the Disneyland of healthcare.” Words can’t really describe the impact of all of the amazing art and healing that goes there.
It has to be experienced in person. However, I will do my best to share with you here some of the things that I find bring so much light to a place that people go when they are facing the most serious situations of their lives.
Mayo Clinic has an art collection that is second to none. You can take a docent led tour if that fits your schedule. These tours are on weekdays at 1:30 p.m. If you can’t fit that in, there is a self-guided audio tour — you simply pick up a handset at the information desk. Or, you can do what I did: pick up a pamphlet and just walk around on your own.
Here are a few pieces that I liked.
Welcome, a kinetic sculpture by Yaacov Agam, rotates constantly in the Mayo Building
Off on His Own -Ceramic sculpture by Maggi Giles in Mayo Building
MAYO CLINIC CENTER FOR THE SPIRIT
Another place that you might want to visit, especially if you are dealing with some difficult health challenges, is the Mayo Clinic Center for the Spirit; you can go for some quiet time to center yourself, or communicate with a higher power of your choice. You can even leave a message in the Prayer Wall.
If you have more time, you should stop at the historical exhibits in the Mayo Clinic Heritage Hall, where you can see the first heart/lung machine. And there’s a bigger, fascinating museum in the Plummer Building next door.
VISITING ST. MARY’S HOSPITAL
After I finished visiting the downtown Mayo Campus, I went over to St. Mary’s Hospital, where I had my open heart surgery 12 years earlier.
The admissions office you see below was where I had to report at 5 a.m. the morning of my surgery.
During the hours I was in surgery, my husband waited for me in this waiting room. Every so often, someone came to find him to give him a update on my condition.
When I woke up after the surgery, I found myself in an ICU room which looked just like this one.
A day or two later, I was moved to the step down unit on Mary Brigh.
Recent myectomy patients will recognize these ducks which can be found around the unit. The ducks arrived a few years after I had my surgery, but most patients count them as they walk laps around the halls on their road to recovery.
No visit to St. Mary’s would be complete without a visit to my favorite cardiac surgeon, Dr. Joe Dearani. Dr. Dearani literally gave me my life back when he removed the piece of muscle that was obstructing the flow of blood out of my heart.
Before I returned downtown, I went to see the chapel – a beautiful old church which is home to the sisters of St. Francis.
Below you can see the tenets of care that were first established by the Mayo Brothers. And as anyone who has ever been treated at Mayo can attest, they really adhere to these principles! Every time I visit, I continue to be amazed at the quality of care I receive at the Mayo Clinic.
And one day not too long from now, I will once again return to Rochester. Until my next visit, Mayo Clinic!
For more about the Mayo Clinic HCM experience, check out these older posts on HCMBeat:
The software update which allows the Apple Watch 4 to take an EKG and to detect atrial fibrillation went live last week. In anticipation of the availability of these functions, I purchased an Apple Watch 4. As soon as the software was available, I downloaded it and have used it every day since. So far, I am quite pleased with my purchase. The technology works very well, even despite the fact that I have an implantable pacemaker/defibrillator.
The strip it takes looks like this:
You can send a strip via email to your doctor, and all are saved for posterity on your Iphone. (NOTE: YOU MUST HAVE AN IPHONE CAPABLE OF RUNNING THE SOFTWARE IN ORDER TO USE THE WATCH).
And, as long as you tell the software that you have never been diagnosed with atrial fibrillation, if it detects atrial fibrillation while you wearing the watch, it will send you an alert. I haven’t gotten such an alert yet and hope not to!
This article provides a pretty accurate history of handheld consumer EKG devices along with a description of what it is like to download and use the Apple software.
And here is a story about a man whose watch spotted his previously undiagnosed Afib. After a trip to the emergency room, he was able to receive proper treatment and avert a potential health crisis.
In this conversation, Drs. Jacoby and Papoutsidakis emphasized that the shared decision making process is an important facet of the patient/physician relationship for HCM patients. Risks should be explained, and decisions made with each patient’s set of values and priorities in mind. The doctors hoped that the results from their study would help to inform the shared decision-making process as applied to activities that involve any amount of patient risk-taking.
After analyzing data from 633 patients (282 men and 351 women), 556 patients reported participating in thrill-seeking activities, while 331 continued such participation after their HCM diagnosis. The doctors found that only 33.6% of the patients who engaged in the thrill-seeking activities experienced such minor adverse events as dizziness, nausea, palpitations or chest pain, while only .02% experienced significant events during or within an hour following the activity. Only one ICD shock was reported.
MyoKardia is collaborating with 23andMe, a genetic testing company which provides ancestry and health information directly to consumers, to create an online patient community intended to advance research efforts related to hypertrophic cardiomyopathy. The companies plan to allow 23andMe customers access to the latest information about HCM, as well as the opportunity to participate in research.
The companies will use a custom designed survey to collect baseline and follow-up data from HCM patients. They are hopeful that this collaboration will yield unique insights into HCM.
Research findings gained through the collaboration will be shared with HCM patients through the 23andMe platform. Currently more than 6,000 HCM patients are customers of 23andMe
DISCLOSURES: HCMBeat has received unrestricted educational grants from MyoKardia. Additionally, Cynthia Burstein Waldman of HCMBeat serves as a Patient Advisor on the Steering Committee for MyoKardia’s Explorer trial.
This study examined patients from eight high volume HCM centers which aggregated their institutional data into a database known as the Sarcomere Human Cardiomyopathy Registry (or the acronym the “SHaRe” for short). The results of the study showed that, in general, 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.
The SHaRe Registry centers that participated in the study are:
Brigham and Women’s Hospital, Boston
University of Michigan Medical Center
Stanford University Medical Center
Boston Children’s Hospital
Yale-New Haven Hospital
Careggi University Hospital, Florence, Italy
Erasmus University Medical Center, Netherlands
Laboratory of Genetics and Molecular Cardiology, Sao Paulo, Brazil
Working together, these centers, led by Dr. Carolyn Ho, M.D. of Boston’s Brigham and Women’s Hospital, made some significant findings.
Patients with HCM Genetic Mutations Fare Worse: HCM patients with a known genetic mutation were diagnosed with clinical disease at a younger age (37.5 years, compared to 51.1 years for patients without a mutation) and were more than twice as likely to experience HCM-related complications and early death than HCM patients who had a non-genetic form of HCM. Patients with more than one mutation and those who carried a MYH7 mutation were found to have a higher risk of HCM related complications than those with single mutations or those with a MYBPC3 mutation.
HCM Burden Increases Over Time: The burden of disease and complications increased progressively over time for HCM patients, with most HCM-related complications occurring later in life between the ages of 50-70 years. In particular, the researchers found that patients who were less than 40 years old at diagnosis had a 77% chance of having an adverse incident such as a cardiac arrest, heart failure, atrial fibrillation, stroke, or death by the time they reached age 60. The most common complications were heart failure and atrial fibrillation. In contrast, patients diagnosed with HCM after the age of 60 years of age had only a 32% cumulative incidence of such complications by age 70 years.
HCM Mortality is Significant: Mortality among HCM patients was found to be significantly higher than that of the general U.S. Population. In fact, among young HCM patients ages 20 – 29, mortality was found to be four times higher than that of their healthy counterparts.
The lead author of the paper, Dr. Ho, suggests two major takeaways from this research:
1. A young age of diagnosis and the presence (or absence) of sarcomere mutation(s) should be taken into consideration when forming treatment plans.
2. Given that the majority of HCM complications occur later in life, there is need for long-term care and follow-up of HCM patients, as well as for the development of new therapies that prevent long term complications such as heart failure and atrial fibrillation.
Dr. Maron discusses the many advances that have been made in the diagnosis and treatment of hypertrophic cardiomyopathy since it was first described 55 years ago, noting that life expectancy and qualify of life have dramatically improved in this period of time. According to Dr. Maron, the contemporary management paradigm for HCM have reduced “the risk of adverse cardiovascular events and death to levels below the levels among patients with other cardiac or non-cardiac disorders.”