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In March of 2008 I began treatment for a potentially fatal heart arrhythmia whose cause was unknown. I was fit and healthy, having spent a good part of my adult life as an elite cyclist, much of it racing in Europe.
After 5 years of fence-sitting on the part of my doctors, I have been given a diagnosis of ARVD/C (Arrhythmogenic Right Ventricular Dysplasia / Cardiomyopathy). I lack the known genetic markers for the disease as well as some of the structural changes that accompany ARVD/C, and my condition has shown no indication of being progressive (yet).
There is a growing population of current and former endurance athletes who end up with acquired arrhythmias, and a diagnosis of ARVD/C. But by placing these athletes in the ARVD/C patient population, we are potentially ignoring the very real likelihood of an acquired condition, similar, but different, from ARVD/C.
This blog is written as a resource for athletes with acquired arrhythmias, as well as anyone about to undergo endo or epi-cardial ablation, or the implantation of a ICD. It is also written to help generate some critical mass of interest in support of further research into acquired arrhythmias in endurance athletes and others. Feel free to contact me through the comments page if you have any questions or wish to make a written contribution to this blog.


Tuesday, March 17, 2009

To "D" or not to "D"?

"Dysplasia": the cardiac wall of an ARVD victim...(if this disturbs you scroll down to the happy wombat).

Arrhythmogenic right ventricular dysplasia / cardiomyopathy (ARVD/C) has been discussed in passing on this blog before . The Wikipedia page is a decent, if a bit technical description of the disease, the Cleveland Clinic has a more accessible layman’s explanation, and Johns Hopkins ARVD Center has more detailed information including the complete clinical diagnosis guidelines. There is also an excellent information clearinghouse and support group at http://www.arvd-arvc-info.com/.

In brief ARVD/C is a form of cardiac disease in which the heart muscle of the right ventricle is replaced over time by fat and/or fibrous tissue. The reason why ARVD/C keeps popping its head up here is that I am, symptomatically, a likely case of ARVD/C. To an ARVD/C specialist my ECG reads suspiciously, and my experiences leading up to my year-long heart saga are textbook ARVD/C signs and portents.

The “D/C” tacked onto the “ARV” seems, in many ways, to be indicative of the nuance required in diagnosing this disease, though the actual reason for the "Dysplasia / Cardiomyopathy" dual moniker is one of semantics. From what I have been able to discern different people in different places use the term interchangably, with no differential in the diagnosis. Dysplasia refers to an abnormality in the way cells replace each other as a body ages. As a dog person I always think of dysplasia in the context of the hip dysplasia common to large breeds. Cardiomyopathy is a general term for any disease of the heart muscle. Unlike dysplasia, cardiomyopathy does not imply a progressive deterioration.

So the question that has lately loomed over my head, and might or might not be answered by the results of my forthcoming genetic testing is this: is my condition progressive (i.e. heavy on the “D”) and is my heart turning into a fatty fibrous lump, accelerated by the fact that I continue to exercise? Or is my condition stable over time, and I merely have to learn to live with my ICD and the off-chance that some of the already-existing ablated scar-tissue might come back “on-line” as a dangerous arrhythmia.

My electrophysiologist Dr. Marchlinski subscribes to the latter theory. He has been, and continues to be, hesitant to give me an ARVD/C diagnosis. Initially, after my first visit, ARVD/C was a likely diagnosis, and I was advised according to the protocol for this disease, which includes a total moratorium on any exercise more strenuous than walking. But as tests came in, including repeat echocardiograms an MRI, angiogram, and the more invasive electrophysiology test, Dr. Marchinski grew more positive about my likely outcome. After my first ablation he declared that, once my arrhythmias were under control and my ICD installed, there would be nothing I couldn’t do (baring contact sports that might rattle the ICD).

This flies in the face of the more conservative approach to a “possible” case of ARVD/C. Regardless of my testing “clean” of any fatty fibrous tissue replacement beyond the pre-existing scar, it seems that my continuously funky EKG and past history of fainting and V-Tach is enough to imply a potential for long term progression that should be heeded. The ARVD/C specialists, in other words, say I’m damaging myself by continuing to exercise.

So my task as it stands is to triangulate all this information and decide how much, if any, exercise is going to be appropriate for me. Because the current gamut of opinion coming from extremely competent practitioners runs from “don’t worry, do as much as you want” to “you are doing permanent damage to yourself if you don’t stop”, this is pretty heady stuff. It also makes it clear just how inexact a science / art medicine is.

4 comments:

Anonymous said...

Hi Craig - I came across your blog last week as my husband was in the hospital recovering from his 8th ablation. He was diagnosed in March 2006 with ARVD. He was 59 years old, involved in fitness all his life, mostly weight training and competitive bodybuilding, came home from work as a police officer and had some chest pains due to an irregular heart rate of 240. He was transported to the ER where he converted back to normal rhythm without shock and after a few trips to the cath lab was diagnosed with ARVD. There begins the long story from the past three years. He had an ICD placed back in 2006 and has not been incident free. Actually he has not been able to go more than five months without incident since then, despite the fact he's had numerous ablations. I've always wondered about his ARVD diagnosis, he just didn't fit the criteria. However, there isn't any other explanation at this time for his disease. We're not sure about the progression at this point. Anyway, this is the very short version of a long story. I just wanted to send you a quick note, to say hello, not too many people out there with living with this disease - ironically I'm the cyclist in the family! Good luck and I'll continue to read your blog for updates. Karen

lunalander said...

Dear Craig,
I don't know if Dr. Marchlinski is right or not.
I'm not an expertise It's sure that all strenuous exercise (excluded low speed walking on flat) cause blood pressure increase during the exercise itself.
The study of my friend Francesco (not a Dr. at all) shows that only exercises at minimal specific muscular contraction guarantee no blood vessel constraints, that means low blood pressure.
(Like for a mechanical membrane pump when we increase the pressure losses, for example squeezing the output pipe).
How to show that?
Give a look to this video: http://www.youtube.com/watch?v=tOyrm4tl2ck
Only to show what I mean for a decontraction state.
This is not a definitive answer but it should be an occasion to start a positive discussion around an efficient and clever use of the human body.
Experts in heart deseases and rehabs should properly answer to this question.

Good luck.

VF said...

Craig,

I have read your entire blog and find your condition somewhat similar to mine, except I've experience SCD and 5 shocks from my ICD for sustained VF events. My docs have no explanation for my condition - idiopathic VF - I don't really get a chance to feel my heart race other than feeling faint, as my bpm shoots from 110 to 300 triggered by 1 PVC. To make matters worse my docs have conflicting opinions as to whether an ablation will prove successful. I'm now loaded with b-blockers and restricted (endlessly) to activity that is under 110bpm....so painful.

Like you I'm searching for answers as to why someone that can pull 13.6 mets on a stress test with a max hr of 210 and no other physiological issues and a healthy heart with an EF of 66 suddenly becomes crippled with this problem. I found the training while sick issue interesting, as I often trained on college crew while sick.

Though your trek wasn't without challenges, it is promising that you were able to resume a somewhat active lifestyle. I hope all is still going well for you - any updates?

Jim Dowling said...

Hi Craig,
I'm 35 years old and have ARVD/C. I had my first PVC at 20. Got it checked out, no big deal, I only got the odd PVC at 170+ bpm which I could handle.
Then when I was 32, suddenly I started getting PVCs at 140 bpm. I pushed myself, went into v-tach, and got it checked out.
In the last 3 years, I've trained - like you said. My diagnosis, like yours, was suspected ARVD/C. Ejection fraction 60+%, no structural damage on MRI, only delayed potentials on signal-avgd EKG. So i didn't meet the ARVD criteria, my doc said - exercise away. So I did. Two years later, I wish I hadn't. Now I get PVCs at exercising at 100 bpm (or less if i exercise for a long time), and even get PVCs at rest. ARVD is progressive, and if you exercise you make it worse and it doesn't get better. I was also crazy about sport, but luckily I have other interests that keep me busy now.