I felt prepared because I read posts and blogs from other patients, conducted a lot of research, and felt informed going into this.
Editor’s Note: A few weeks ago I had the chance to sit down over Skype to talk with Yale University Medical Center’s Dr. Daniel Jacoby and Dr. Nikolaos Papoutsidakis about HCM patients who engage in thrill-seeking activities.
Drs. Jacoby and Papoutsidakis are currently circulating a questionnaire via the internet in connection with a study they are engaged in related to the safety of these activities. They are hoping to obtain at least 500 more responses to their questionnaire from HCM patients around the world in the next two months. Keep reading to learn more about the study, and if you want to participate, a link to the survey is provided at the end of this article.
Are you a HCM patient who has ever wondered whether it was safe to ride on a roller coaster? Well, it turns out that this question is one that HCM doctors encounter quite frequently from their patients. This question and the lack of empirical evidence about its safety inspired doctors at Yale School of Medicine and Yale New Haven Health Heart and Vascular Center to design a study to answer the question whether thrill-seeking activities are safe for HCM patients.
The literature about HCM is full of warnings against activities thought to be unsafe for patients with the standard response being “No!” With the knowledge that patients ARE actually out there engaging in such activities, Drs. Jacoby and Papoutsidakis decided to go out to the internet and crowdsource more information.
Having already surveyed patients at Yale, the doctors sought to generate more data – their goal is at least 500 additional patients in the next two months – by crowdsourcing enough data to enable them to put together a statistically significant sample which could be written up in a medical journal. This information would serve as a resource to doctors caring for HCM patients who could bring data to the conversation with patients when asked about rollercoasters or other thrill seeking activities.
The activities chosen for the study’s questionnaire were specifically chosen from previously published literature describing high risk activities believed to be unsafe by most practitioners.
These activities are:
- Rollercoaster riding
- Jet Skiing
- Bungee Jumping
- Motor Racing
- BASE jumping
The questionnaire also seeks to learn what lifestyle information has been provided to patients by their cardiologists. As an adjunct to the study about thrill-seeking, the researchers also plan to examine whether there is a difference between patient lifestyle and/or overall health when treated by a community-based general cardiologist versus a HCM expert.
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 hope that the results from this study will help facilitate the shared decision-making process as it applies to activities that involve any amount of patient risk-taking.
Dr. Jacoby knows the importance of shared decision making between physician and patient from personal experience. He and other members of his family have a genetic condition known as hypercholesterolemia, a condition that can cause early heart attacks. This gives him unique insight into what it means to be a cardiac patient and provided his young self with an unpleasant awareness of the fact that people may die early from heart issues. This, for him, was an eye-opening experience and gives him a unique ability to understand and empathize with HCM patients who face similar issues every day and informs his professional goal of helping his patients live their best and most fulfilling lives.
Both doctors shared that although some might think that riding on rollercoasters or participation in extreme sports may seem to be a frivolous or unnecessary subject for scientific study, this is actually an issue that may genuinely impact the quality of life of certain patients. Drs. Jacoby and Papoutsidakis want to help patients feel more reassured about engaging in these activities so that they can continue to enjoy activities that give them pleasure. After all, acceptable quality of life and activities which provide fulfillment are subjective.
About the researchers:
Dr. Nikolaos Papoutsidakis is a research scientist at Yale. He received M.D. and Ph.D. degrees and his cardiology training in Greece at the University of Athens. Around two years ago, he joined Dr. Jacoby’s team as an Associate Research Scientist to work on cardiomyopathy research projects with an emphasis on HCM. Yale patients may recognize him because he often shadows Dr. Jacoby in clinic to keep track of patient data for the Yale Inherited Cardiomyopathies database. He was responsible for the design of the current survey, tracking of responses and correlating data.
Dr. Daniel Jacoby is the founder of the Yale Inherited Cardiomyopathy Program and runs the HCM Clinic at Yale where he treats approximately 500 HCM patients. Dr. Jacoby’s undergraduate and medical degrees are both from Yale. When a fellow at Columbia Presbyterian hospital in New York City, he took care of a young Venezuelan patient who suffered from HCM and this piqued his interest in HCM. After completing his training in NYC, he returned to New Haven to join the faculty at Yale Medical School in 2009. At Yale, he had the opportunity to collaborate with longtime HCM expert Professor William McKenna, then of University College London, through a collaborative program between Yale and the University of London.
NOW THAT YOU HAVE READ ALL ABOUT THE SURVEY, FILL OUT A QUESTIONNAIRE YOURSELF! THE LINK IS JUST BELOW. IT SHOULD ONLY TAKE YOU A FEW MINUTES.
Recently, I began shopping for a new car. The process is overwhelming! There are so many factors to consider when looking for a new vehicle: gas mileage, sedans vs. hatchbacks, SUVs…the list goes on and on. Electrics and hybrids are all the rage here in Southern California, but I wasn’t sure if they would be safe for me to drive because I have an implantable defibrillator which also functions as a pacemaker.
German Study: Safety of Popular European Electric Cars
Lucky for me, I didn’t have to wait too long for an answer to my question. According to a recent German study published in the Annals of Internal Medicine, people with implanted cardiac devices can safely drive the most common electric cars on the market today. This study measured the magnetic field strength in four electric cars with the largest market share in Europe: the BMW i3, Nissan Leaf, Tesla Model 85S, and the Volkswagen e-up! Though the study found that recent models of all of these cars were safe, the authors of this study did caution that future models could potentially cause interference with implantable cardiac devices, depending on their design.
2017 AHA Preliminary Data – Tesla
The findings from the German study added to preliminary data presented at a 2017 meeting of the American Heart Association.
Participants’ devices were monitored for electromagnetic interference while they sat in or stood near a Tesla S P90D. Testing was done with the study participants situated in a variety of positions—sitting in the driver’s seat, passenger seat, backseat and standing next to the charging port.
The study found that sitting in, or standing close to the charging port of a Tesla while the car was charging at a 220 volt charging station did not trigger an ICD shock or cause interference with the assorted implantable defibrillators.
2013 Mayo Clinic Study – Toyota Prius
That early study found no issues when patients implanted with ICDs and/or pacemakers drove a 2012 Toyota Prius hybrid at 30 mph, 60 mph and at variable speeds of acceleration and deceleration, as well as sitting in the driver’s seat, the front passenger seat, the left and right rear seats and in front of and behind the car from the outside. Although the researchers found that the implantable devices were exposed to electromagnetic fields inside the car, the amount of interference wasn’t significant enough to cause problems with the devices.
For more on the 2013 Mayo study, see this article in Popular Science.
Now that I know that driving these cars is safe for me, I will be out on a test drive trying to narrow down my options!
A retrospective study of HCM patients with implantable defibrillators conducted at eight centers worldwide has demonstrated that ICDs are not only lifesaving, the shocks they generate are not harmful to those in whom they are implanted.
The study looked at 486 patients with HCM with an ICD implanted for either primary or secondary cardiac arrest prevention. Of the 486 patients, 94 (19%) experienced at least one appropriate shock from their ICDs. 44 of those who had been shocked had experienced one or more shocks over the period of the study, including 6 patients who had at least 3 shocks over a 24 hour period. Inappropriate shocks occurred in 96 patients (20%).
Despite the shocks, appropriate or not, at the end of the follow-up period the ICD discharges did not appear to cause the patients to suffer from increased heart failure or sudden cardiac arrest. Furthermore, their general health and well-being were good: they did not suffer from significant degrees of anxiety and depression.
A recent editorial published in Circulation: Genomic and Precision Medicine suggests that current HCM screening protocols may need adjustment to account for recent findings by a study by researchers in the Netherlands. The Dutch study, published in the same journal, found that of 620 relatives of HCM patients who underwent genetic testing, 43% were found to be genetically positive for HCM, while 30% were diagnosed with HCM at the initial screening. 16% more went on to develop HCM during 7 years of repeated cardiac evaluation.
On the other hand, the 57% of relatives found to be genotype-negative were released from clinical HCM follow-up.
The Australian authors of the editorial, Semsarian and Ingles, note that current screening protocols would have failed to identify the 6 children (15%) who were diagnosed under the age of 12, half of which had a particularly malignant family history.
Additionally, few teens were diagnosed with HCM, which stands in contrast to current opinion that HCM is most likely to develop during adolescence. Indeed, most newly diagnosed family members were older than the age of 36, with 44% being over the age of 50.
Lastly, Semsarian and Ingles note their concern with general utilization of the Dutch practice of releasing a gene negative family member from serial follow up since the impact of all genes which have a role in causing HCM is not yet known while new genes which may cause HCM are still being identified.
Semsarian and Ingles also note that the Dutch patient sample differs from more typical patient populations found in the U.S. and Australia where causes of HCM are more diverse and cannot be easily tied to a specific gene.
A recent article by doctors from the University of Utah Health Sciences Center found that patients with HCM who are in need of heart transplantation may wait longer for a new heart than patients with other cardiomyopathies. Additionally, HCM patients may experience stroke or other adverse consequences while awaiting transplant.
The discrepancy is attributable, in part, to the fact that HCM patients are not often candidates for LVADs (left ventricular assist devices) and other types of mechanical circulatory support devices which are used to bridge patients awaiting transplant.
Hence, the article argues, United Network for Organ Sharing (UNOS -the organization responsible for the allocation of donor organs in the U.S.) should take these factors into consideration as it revises its system of heart allocation for patients awaiting transplant.
On a positive note, the article points out that long-term survival in HCM patients has improved over time, and HCM patients now do as well or better following transplant than patients who have been transplanted for other types of cardiomyopathy.
This recent article by Dr. Srihari Naidu of New York’s Westchester Medical Center uses great detail in describing the technique used to perform alcohol septal ablation in treating obstructive HCM.
The article also describes what is involved with the newer, experimental Mitra-Clip procedure, and discusses appropriate patient selection for both procedures.
The January recall of 48 Medtronic CRT-D and ICDs has now been expanded to include 752 additional devices at lower risk than those involved in the January recall,. There is an issue that occurred during the manufacturing process of these devices which could result in an unexpected loss of device functionality. If you have one of these devices, you should contact your doctor to discuss next steps.
You can see the advisory here.
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 you are affected, the search page would look like this:
This example shows that currently there are no advisories for my model.
As always, you can call Medtronic Patient Services with any questions at: (800) 551-5544 (M – F, 8am – 5pm Central)
Medtronic has recalled a small number of ICDs and CRT-D devices. A total of 48 devices implanted in patients may contain a manufacturing defect which would prevent the device from delivering an appropriate shock if needed.
This is a Class I recall, which is the most serious as determined by the FDA.
Physicians of record of those affected by the recall should have already been notified by Medtronic.
You can also contact Medtronic Patient Services at 800-551-5544 (Monday-Friday, 8am-5pm Central Time).
The article focuses on the motivation for and the impact of HCM genetic testing on family members. The 32 participants in the study all encouraged family members to undergo genetic testing with the hope that the knowledge gained would benefit family members down the line. However, the study found that the psychological impact of a positive result, in the absence of overt disease, was highly variable. Some gene positive individuals perceived that they had an absolute risk of developing HCM, with substantial detriment to their lifestyle choices, while others were not at all affected by the result and made no lifestyle changes.