Off To See The Wizard….

     It’s been a while since my last cardiac update and it’s nice to hear from others who are interested in ARVD or have the misfortune of being in the same boat. It’s been 9 months since my epicardial surgery and I haven’t had a run of V-Tach since. I met with my cardiologist who was very pleased to hear the news and over the course of the winter we went through the battery of tests required by the 2010 Task Force Criteria for ARVD. (I think they might be working on new criteria but currently the 2010 version is the standard).

     So what is ARVD? Ask your doctor or visit But for people with no idea and just want a basic notion, I’ll take my exiguous view and dumb it down even further just for fun…

You probably recall on the first day of school in your first anatomy class the teacher said:

Welcome class! The human heart is a muscle!– your science teacher

…while you scanned the room to see if any number of your secret crushes also happened to be ensnared in the same educational cell. In simple terms, if you have ARVD your heart slowly morphs from a muscle that pumps efficiently on demand into a big hunk of mushy, lazy fat. It’s thought that exercise is bad for people with ARVD. Newly diagnosed patients typically receive complementary crotchet needles and 5 gift certificates to the nearest shuffleboard parlor in a parting gift bag. The theory is that exercise strains and tears the heart muscle (just like you would strain you muscles at the gym) and for 99.999% of the population this is a very good thing. The heart rests and adapts itself so the muscle is stronger and more efficient for the next bout. In a person with ARVD, it is thought that the muscle tears are not rebuilt, but rather replaced with the dreaded fatty tissue. Heart functions are compromised as it cannot pump blood efficiently and oxygen is depleted from the cells. The circulatory system struggles with the inadequate cardiac performance causing shortness of breath, fainting, and sometimes sudden cardiac death. While the fatty demise of an ARVD heart is currently inevitable, it’s thought that exercise will increase the speed at which the fat takes over and commands the ship. Patients with ARVD can choose to have a ICD implanted to control any arrhythmias which can increase the chances of SCD. The prognosis of someone with ARVD can be quite good. Catching it can reduce the possibility sudden cardiac death and one can live a long life with ARVD, provided you slug though life at a miserably slow and sedentary pace.

     Diagnosing ARVD is difficult and is changing as more is known about the mutation. It’s thought to be genetic and part of the Task Force Criteria includes screening for known genetic sequences associated with ARVD. Unfortunately, a positive genetic test is only prevalent in 50% of the known cases of ARVD. Other factors are similarly vague with plenty references to ‘sometimes’, ‘could be’, ‘sorta’, ‘if this and that then maybe’, and ‘flip a coin’ in the medical journals. (Actually the journals phrase this slightly differently, but it would be more entertaining if they adopted my nomenclature then the silly medical acronyms and shorthand). In response, a Task Force Criteria was established that roughly works on the ‘3 strikes and your out’ maxim. Currently, there are 6 criteria that you can either fail in a ‘Major’ or a ‘Minor’ way. After all the tests are completed, a diagnosis of ARVD is:

  • Definite: 2 major OR 1 major + 2 minor
  • Borderline: 1 major + 1 minor OR 3 minor
  • Possible: 1 major OR 2 minor

Although the criteria could easily be confused with the rules to an obscure children’s game, it’s about as scientific as one can get in 2014.

     My score was 3 minor – borderline but not definite. I don’t have any of the classic red flags such as fainting or a positive genetic test and my ECG and cardiac MRI do not point directly to ARVD.

     I’m still in the game, standing at the plate, but the count is full. I was able to bike more this winter than the previous 2 years and spring riding has been going fairly smoothly. My cardiologist has dismissed the idea of diagnosing me with ARVD as he correctly followed the Task Force Criteria. He presented me with the idea of traveling to see one of the 3 doctors in the world who focus on ARVD and I’ve decided to sling a bindle over my shoulder and follow the Yellow Brick Road to Johns Hopkins in Baltimore to get some answers. In addition to going over all my past tests, I’ll again be spending 2 hours trying not to move a muscle while holding my breath in an MRI tube followed immediately with a full-throttle stress test on the treadmill. Now that I think about it, this seems rather taxing on a normal heart. The appointment is Friday. Anyone seen my oil can?

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2 Responses to Off To See The Wizard….

  1. arvdheart says:

    It’s been a while since you posted about cardiac and ARVD stuff, but I see you’re posting again albeit sporadically about cycling so I’ll take that as a good sign regarding your health. Thank you so much for this fabulous post. Like you, I love some good science and the tech piece of having an AICD (all mine have been Medtronics) is fascinating.

    I have a novel Desmoplankin mutation that resulted in a sudden cardiac arrest 17 years ago, and killed my brother 20 years ago. We were both in our 20s when the events happen and had no forewarning, both in great shape and were running at the time (see there’s the problem- running!) My cardiologist following the event was the guy who first described ARVD in the 80s, but I didn’t test positive for the mutations they were looking for at the time and so when he semi-retired I got booted from his patient list. Just a bit of reassurance for you re: the exercise piece. I’m a definite on the ARVD, with an additional mutation that maybe just adding to the whole beauty of the matter. For 17 years they told me to exercise, just not to go to max HR, and while I’m no athlete, indeed I’m overweight now but that’s another story, I was active for many years after the event, skiing,kettlebells swimming, hiking etc. The progression of my disease in the first 15 years (I’ve just had an echo don’t know about those results yet) since my sudden cardiac death has been very slow, pregnancy 8 years ago seems to have had an impact, but that shouldn’t be an issue for you. My cardiologist is hopeful that progression will continue slowly, knowing of course that this disease appears to progress at different rates with limited rhyme or reason. Here’s hoping you never need your ICD and to many years of happy and wayward cycling. PS even since the positive gene mutation they’re saying to exercise for exactly the reasons you describe. Best of luck

    • thewaywardcyclist says:

      Hi ARVDHeart,
      It’s always tough when genetics are not in your favor. Your story seems like the ‘classic ARVD/C’ story that you hear with a family history. It’s interesting that they said exercise was ok – as the classic ARVD with family history puts all exercise to a halt. It would be interesting to know if the ARVD community can come to a consensus on an acceptable level of low exertion exercises that wouldn’t tear the heart muscles to progress the disease, but still provide adequate exercise for one’s general health. Have you looked into the Annual John’s Hopkins ARVD Symposium? It’s open to everyone and I’m sure they would be interested in a topic such as this if they have not answered it already. It’s typically held in late May in Baltimore.

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