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The following question was recently directed to ARVD-ARVC-Info.com:

"What I am looking for are definitive, clinical based outcomes and recommendations for individuals who have been diagnosed with ARVD as to what exercise is allowed and what is not recommended."

• Dr. Frank Marcus:
Principal Investigator, Director, NIH Multidisciplinary Study of Right Ventricular Dysplasia
University of Arizona, Sarver Heart Center, Arizona, US

• Dr. Srijita Sen-Chowdhry
The Heart Hospital, London, UK

Compiled by: Micheline Long
Date: July 16, 2008
Contributing Authors: Micheline "Tink" Long, Dr. Frank Marcus, Dr. Srijita Sen-Chowdhry
© Copyright Micheline Long 2008

Hello [a],

You wrote:

"What I am looking for are definitive, clinical based outcomes and recommendations for individuals who have been diagnosed with ARVD as to what exercise is allowed and what is not recommended."

Dr. Hugh Calkins: hcalkins@jhmi.edu
Crystal Tichnell: ctichnell@jhmi.edu
Dr. Frank Marcus: fmarcus@u.arizona.edu

As far as I know, there are no clinical studies on ARVD and exercise, although I could be wrong. There may be some retrospective studies, but I am not aware at the moment.

Question: Has there been a study on Exercise and ARVD?
Answer: YES! Thank you Dr. Budzikowski! [Click here to read the abstract.]

I believe that the researchers point out that this is a disease of the desmosome, the "glue of the cell," so to speak.

"The newest 'kid on the block' ARVD/C, is a disease of the desmosome that is responsible for intercellular binding." *

The genetics seem to point to the cells of the heart breaking apart, at which point fat/fibrosis is thought to form. I believe that it is thought that the engorging of the heart with blood, during exercise, stretches the heart and compromises cells which are affected by a genetic mutation which has already compromised them.

Sometime ago I put a document together called "Exercise and the ARVD Diagnosed Person" (ExpertsOnExercise.html) Within that document you can see what a number of the researchers have said. I believe I updated that document in 2007. In the 2006 update (see the reference at the top of the page in ExpertsOnExercise.html), there is information about a mouse model study. At the time the information had not yet been published, but perhaps by now it has been.

Click here to go to our "Patient" library and find/read the ExpertsOnExercise.html

Within the above "Patient" library, there is another document:
ARVDandSportsActivities.html

The ARVDandSportsActivities.html refers to the article, Recommendations for Physical Activity and Recreational Sports Participation for Young Patients With Genetic Cardiovascular Diseases (Maron et al; Circulation. 2004;109:2807-2816)

I "think," though I am not sure, that Italian ARVD researchers have really looked into athletics and ARVD. There is a Dr. Domenico Corrado who appears to have some information on this. Perhaps Dr. Calkins, Crystal, or Dr. Marcus could guide you to a contact with Dr. Corrado or one of his colleagues who has looked into the matter.

There is a document from Dr. Corrado et al (those on the Italian ARVD research team.) It is an older, but valuable document.*

* Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment (Corrado et al, Heart 2000:83:588-595)

In giving a brief explanation of the phases of ARVD, the above mentioned article notes the "concealed phase" of the disease. Within the text, there is a fairly sobering comment about the concealed phase of ARVD, competitive sports and intense physical exercise:

"'Concealed' phase characterised by subtle right ventricular structural changes, with or without minor ventricular arrhythmias, during which sudden death may occasionally be the first manifestation of the disease, mostly in young people during competitive sports or intense physical exercise."

"ARVC is found to be familial in 30 to 50% of the cases."*

Amidst the ARVD diagnosed community, the question arises, "What are the recommendations for exercise and sports for my children and others in my family who may have inherited the ARVD gene mutation?" Long time ARVD researcher Dr. Frank Marcus addressed this concern and recommendation in his 2006 update found in the ExpertsOnExercise.html document (mentioned above):

"This information reinforces the recommendation that competitive exercise should be avoided not only in those affected with the disease, but also in family members who have the gene defect but have no clinical evidence of structural or functional abnormality of the heart."

At this point, my experience is that many ARVD diagnosed patients who still feel that they must exercise are walking (as advised), doing easy swimming (shallow water), playing golf, perhaps doing easy kayaking, doing Pilates and/or Yoga (being careful with stretching, due to their ICDs/wires), lifting light weights to tone... Some are doing a bit more strenuous exercise like rock climbing, or light jogging, although both of the latter may not be recommended -- I don't know.

A consistent question amidst the patient community is "Under what heart rate should I stay when exercising?" Of course, for some this depends upon the rate at which they will be shocked by their ICD, some having very low settings for this. This question of keeping heart rates at a certain level does not directly address at which rate a heart might have its right ventricle stretched and thus compromised. For some with ARVD, it is enough that they can do normal daily activity and enjoy intimacy, some are entering CHF (congestive heart failure), others want to avoid it.

A challenge for some with ARVD is what they actually can do without compromising their heart or bringing on a shock. They know that being a "couch potato" is not recommended, although some with ARVD can bring on an arrhythmia in an unstable period and with little more than walking from the couch to the shower. BTW, the patient community has learned that getting overheated (too hot and/or too humid i.e. in hot tubs, swimming pools, hot showers, out doors doing normal activity such as mowing a lawn, etc.) can often bring on an arrhythmia. Many absolutely know that they MUST avoid getting too hot. Similarly, a number of them have learned a danger in getting too cold, although this is probably lesser seen. Many with ARVD absolutely gear up to avoid the summer heat and/or humidity.

Some patients have found some benefit in going to "the right" Cardiac rehab program to learn their limitations, relieving their fear about what they are able to do in normal life. A "right" program might be one wherein the techs were well aware of what the experts say to avoid in ARVD. Some years back, an ARVD patient was hugely compromised in a program when they were being forced into step dancing which was a very wrong exercise for this ARVD patient. I believe that start/stop exercise (like racquetball, tennis), as well as types of weight lifting are advised against (perhaps those which bring the BP up too quickly).

Finally, in the event it can affect your thinking in designing an exercise program for those with ARVD, perhaps the following anecdotal information will assist. Four patients are mentioned.

After one of the many many discussions about exercise in our ARVD support group over the course of 9 years, the following message came forth. It was written by the wife of an ARVD diagnosed patient. The patient was once a Triathlete (half Ironman, Duathlons, Marathoner.) He is now in his late 50s and experiencing a very difficult and extremely diminished life quality. "X" is the husband/patient. "Y" is a female, an ARVD diagnosed patient, a past professional athlete (Triathlons, Ironman, Duathlons). "Z" is a male, an ARVD diagnosed patient and past exercise enthusiast.

From the wife of "X," written in 2008:

"X is, as I write, being transferred to the Y Hospital. He is extremely anxious, and I realized why last night, when visiting him. He will have to come off his meds prior to the ablation, and he is terrified of more shocks. There's not a lot to say to that, because, yes, it is likely. He still has the shaking at times. Not like him at all.

The good old exercise issue rears its head with regularity I note. X and I were talking last night about the insane training he used to do, he said that on bike time trials he pushed himself so hard that he used to be almost unconscious. I'm sure many will identify with that, particularly Y, and Z and I were talking about his "eyeballs out" biking. The thing is, X said he honestly thought that he was making himself fit for life. The irony.

X had a message for all of you, probably younger members, who feel ok, so much so that they are still exercising.... He says, "Please don't do it." If you saw the state that he is in now you just wouldn't take the risk. If you can preserve the state of your heart, then do. Feeling able to do stuff is a fantastic privilege with this disease, and you could be trading years and years of being able to walk where you want, enjoying your life, for just a few years of 'training' (for what exactly?) followed by years of breathlessness, VT, shocks, and feeling terrible. Its a no brainer. Believe me, if there is any chance that you can prevent what X is going through now, take it."

"I was a fit racing cyclist prior to diagnosis and also dabbled in a bit of running (10 mile PB of 58m55sec). I have recently been fitted with an ICD. You'll find a diversity of opinion here with regard to what level of exercise can be tolerated. For me though, I know I can't do very much, and absolutely nothing like what my previous levels of training were. I'd averaged 5000 + miles of cycling for many years, with running on top.

Even if you can run or cycle (or whatever), it's worth bearing in mind the progressive nature of ARVC, and the potential for further damage to the RV in particular due to its pumping action. In my case my RV is, in the words of a cardiologist and following an MRI 'grossly dilated with severely impaired function' Placing additional stress on that would, in my opinion be suicidal. That's my case, everyone is different, but I would suggest extreme caution. I still miss my previous lifestyle, I always will, but at least I'm alive. At no time did I want to be the fittest corpse in the cemetery."

"I was a serious athlete, for several years made my living competing in long events and qualifying for the Olympic Trials three times in the marathon. I have chosen to continue to jog and mountain bike, with no real hard training, and no racing. But my heart is not enlarged, and is pumping quite well. Many do not endorse exercise of any sort that is strenuous. There are ways to exercise without stressing the cardiovascular system, such as yoga and pilates."

"I still exercise. My doctors at the A Hospital and all my local doctors have historically encouraged me to continue to exercise. I am the only one (on my medical team) that decided I should not race anymore, after so many shocks. I do not think that the doctors gave me good advice though, and I wish they had told me to stop racing way back in 2001. I believe I would have listened to them. But at the same time I do not think it would serve me well not to exercise at all. But push ups may not be good for the wires and ICD. There are ways we can all exercise and it is different for us all. Even those that cannot take a brisk walk can find ways to move whilst sitting on the couch or floor! Just my thoughts..."

"I was diagnosed in 20XX at 2X years old. As a young, 'healthy' triathlete the news was quite a shock. ... Since I was diagnosed I have had an ICD implanted (on my second one now). I went through a phase of being very sedentary...mostly out of fear of progressing the disease. Once my cardiologist told me that I was not going to progress this disease and I saw my echos not changing....I allowed myself to move more. I took up walking (a few times I tried running again but that never worked for me...too many arrythmias), yoga, rock climbing.... gradually I added more and more activity. I often think I am healthier now then I was as a triathlete (I trained ALOT). As I allowed myself to start moving again I felt so much better mentally and physically ... and felt better about the future.

My life is very normal and I am more active then most people. I have been blessed in that I am often not too symptomatic. Yes, I take medicine daily and have to have my ICD checked regularly."

Tink

Greetings [a],

I have heard from Dr. Frank Marcus. Dr. Marcus is the Principal Investigator of the NIH Multidisciplinary Study of Right Ventricular Dysplasia (www.ARVD.org).

Dr. Marcus noted that "there are no definitive clinical trials re exercise and ARVC progression." He wrote that this would take many years to do. Further, he mentioned that he wrote an editorial on the subject that was published in Circulation. The editorial is available for reading online at the following link.

The Mystery of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy: From Observation to Mechanistic Explanation (Marcus and Towbin; Circulation 2006;114;1794-1795)

You will see that Drs. Marcus and Towbin put some very good information together in the above mentioned document. Additionally, exercise was addressed in several places. Below is only one quote from the document. It mentions Daubert et al, I've put the reference just below the quote.

"It appears that it takes years and many hundred of thousands of myocardial contractions and mechanical stress forces to gradually disrupt myocardial cells. The right ventricular wall is thinner than the wall of the left ventricle and is therefore more vulnerable to cellular disruption. The thinnest parts of the right ventricle are located in the most vulnerable areas, the "triangle of dysplasia." Sports activities, particularly running and bicycling, produce an increased frequency and vigor of contraction, which facilitate the disruption of the myocardial cells at an earlier age. In addition, the left and right ventricles respond differently to prolonged, vigorous exercise: The left ventricular size is reduced but the right ventricular size increases.6 Further evidence for the adverse effect of exercise on the phenotypic expression of ARVD/C was provided by Daubert et al,7 who found that individuals with ARVD/C who performed intensive and regular sports activities had symptoms at a younger age and that palpitations, syncope, and sudden death were more frequent in the athletic group than in patients with ARVD/C who were not athletically inclined. This may also explain why some members of a family who have a genetic defect of the desmosome but are not athletic may have no phenotypic expression of the disease."

When Dr. Marcus wrote to me, he also mentioned a related article which was published in the same Circulation issue, an article by Kirchhof P et al. He wrote that the latter mentioned article "reported that regular swimming exercise exacerbated the cardiac enlargement in a mouse model of ARVC." The name of the Kirchhof article and the link to getting a copy of it online follows.

Age- and Training-Dependent Development of Arrhythmogenic Right Ventricular Cardiomyopathy in Heterozygous Plakoglobin-Deficient Mice (Kirchhof et al, Circulation. 2006;114:1799-1806.)

In addition to the above, Dr. Marcus also brought to mind that "Sen-Chowdry S et al have a comment about exercise in ARVC published in the Journal of the Amer Coll of Cardiology 2007;50;1813-1821." Below is the document title and a link to where it can be accessed online.

Role of Genetic Analysis in the Management of Patients With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (Sen-Chowdry et al, J Am Coll Cardiol, 2007; 50:1813-1821)

Unfortunately, I do not yet have a copy of the above document so was unable to look up the comment. Perhaps Dr. Sen-Chowdry will have time to share information about exercise and ARVD with us.

Finally, Dr. Marcus wrote, "I advise patients with ARVC to avoid regular physical activities that result in a persistent increase in heart rate such as running, long distance or competitive cycling etc."

I hope that the above proves useful to you in your task, which I presume may have something to do with designing a cardiac rehab program for those diagnosed with ARVD.

Tink

Dear Tink and [a],

Many thanks for your message.

I echo Frank Marcus's comments. The impact of exercise on ARVC remains incompletely understood. At present, two distinct problems are postulated:

1. Exercise is associated with increased sympathetic drive and adrenaline release. Adrenergic stimulation, in turn, may provoke ventricular arrhythmia in ARVC. This is an "acute" problem. Hence avoiding activities associated with high adrenaline levels -- such as organized, competitive sports -- is often recommended, although recreational activies may continue.

2. Existing theories about the underlying disease mechanism commonly invoke mechanical stress as a contributor to cell damage and loss. For example, defects in cell adhesion proteins have been identified in a proportion of patients with ARVC. One can speculate that impaired cell adhesion would compromise the ability of the heart to withstand mechanical stress, leading to cell detachment and death. As already noted below, it would stand to reason that frequent and/or prolonged strenuous activity might contribute to progression of the disease. This is a "chronic" problem and is another reason for patients with ARVC to avoid endurance training.

As per our review in JACC to which Dr. Marcus kindly referred... The comment we make, on page 1817, is that "Long-term endurance athletes appear to have structurally severe forms of the disease...", which in turn is based on our own data, published in Circulation 2007 Apr 3; 115(13): 1710-20, and available for free at the following link:

Clinical and Genetic Characterization of Families With Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Provides Novel Insights Into Patterns of Disease Expression

This was a study correlating clinical, magnetic resonance, and genetic findings in a cohort of 200 patients with ARVC. In a nutshell, we found that markers of structurally severe disease were increased in the eleven individuals who had participated in long-term endurance training (equivalent to >1 marathon per year for >10 years). I emphasize, however, that this is a preliminary finding and large-scale trials would be needed to verify it.

Please don't hesitate to contact me if I can be of help in any way.

Best wishes
Srijita

Edit: 07/19/08

 
Question: Can the screening of athletes help to prevent sudden cardiac death?

Answer: Thank you Dr. Corrado. The answer is YES and YES!

Link to Trends in Sudden Cardiovascular Death in Young Competitive Athletes After Implementation of a Preparticipation Screening Program (Domenico Corrado, MD, PhD; Cristina Basso, MD, PhD; Andrea Pavei, MD; Pierantonio Michieli, MD, PhD; Maurizio Schiavon, MD; Gaetano Thiene, MD; JAMA. 2006;296:1593-1601)

"Conclusions  The incidence of sudden cardiovascular death in young competitive athletes has substantially declined in the Veneto region of Italy since the introduction of a nationwide systematic screening. Mortality reduction was predominantly due to a lower incidence of sudden death from cardiomyopathies that paralleled the increasing identification of athletes with cardiomyopathies at preparticipation screening."

"A recent time-trend analysis of cardiovascular SD of young competitive athletes of the Veneto region of Italy over 26 years showed a mortality decline by almost 90% after implementation of the nationwide pre-participation screening program, whereas the incidence of SD among the unscreened non-athletic population of the same age range did not change significantly. Mortality reduction in athletes was predominantly due to a lower prevalence over time of SD from cardiomyopathies and paralleled the increased identification through pre-participation screening of athletes with hypertrophic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy. All these findings definitively show that pre-participation screening is a life-saving strategy and that the 12-lead ECG is a very sensitive and powerful tool for identification, risk stratification, and management of competitive athletes."

Edit: 08/07/08

 
Question: Has there been a study on Exercise and ARVD?

Answer: YES! Thank you Dr. Budzikowski!

In response to this discussion, we have heard from Adam S. Budzikowski, M.D., PhD., Assistant Professor of Medicine-Cardiology, State University of New York. Dr. Budzikowski is the lead author of the document entitled, Sports participation carries high arrhythmic risk in patients with Arrhythmogenic Right Ventricular Dysplasia (ARVD).¹ He has provided the following abstract and the Fig. 1 to which it links.

"Prior reports in the literature have indicated that sports participation may have association with the onset of ARVD. In this study we sought association between sports participation and arrhythmic events in patients with ARVD. We analyzed 101 patients from the North American ARVD registry looking at prior arrhythmic events, arrhythmia inducibility during programmed electrical stimulation (PES), further arrhythmic events judged by appropriate defibrillator therapy. The level of sports participation was stratified as competitive (COM), recreational (REC) and inactive (IN).

Patients who participated prior to diagnosis in competitive or recreational activity had more arrhythmic events by the time of diagnosis (97 and 81% respectively) than those who were inactive (56% p=0.001). Competitive and recreational sports participation was also associated with a higher frequency of inducibility of ventricular arrhythmias during PES (64 and 25% competitive and recreational respectively vs inactive 13% p<0.001). Only COM had a higher cumulative frequency of further arrhythmic events (Fig 1). We conclude therefore that sports participation at any level in patients with ARVD is associated with higher arrhythmic burden."

¹Sports participation carries high arrhythmic risk in patients with Arrhythmogenic Right Ventricular Dysplasia (ARVD), (Adam S. Budzikowski, MD, PhD[1], James P. Daubert, MD[1], Scott McNitt, BS[1], Wojciech Zareba, MD, PhD[1], Mark Estes III, MD[2], Hugh Calkins, MD[3], Frank I Marcus, M.D.[4], [1]University of Rochester Medical Center, Rochester, NY USA; [2]Tufts New England Medical Center, Boston, MA, USA; [3]Johns Hopkins Hospital, Baltimore, MD [4]University of Arizona, Tucson, AZ, USA.)