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.
Thursday, May 22, 2008
Above is the latest physical manifestation of my condition: the welty burn mark of a defibrillator paddle on my right pectoral.
The backstory: Monday May 19th I was carted off to the cardiac cath lab at U Penn for a follow up electrophysiology study (EP) to determine if the first round of radio-frequency ablation had been a success. Briefly, an EP study is a standard cardiac catheter procedure, in my case the catheter is threaded into a vein through an entrypoint in my groin, it snakes up into the heart and from there it gathers information about the state of things.
For my condition the electrophysiologist "paces" the heart, trying to induce ventricular tachycardia, first with electricity, then with drugs, then with a combination of the two. The hope is that I am un-inducable and then I get to go home with a clean bill of health and start my life again -more or less. That did not happen.
First I produced unsustained V-tach, minor things lasting a few seconds. Next were a few longer runs of sustained V-tach, and finally a seemingly endless run. The last run I was in VT for about 30 seconds when Dr. Marchlinski starting fretting, and ordered the defibrillator prepped. First he waited a bit to see if I would come out of it on my own. As anyone who's watched a made-for-TV medical drama you know there is a specific order of commands before defibrillation. In my case the technician pulled the trigger on the wrong command. I suspect I got hit at the moment I was starting to come out of V-tach naturally. I've been in V-Tach a few times and never had to be defibrillated. Furthermore normally when they defibrilate a conscious person they hit you with sedation right before they defibrillate. My nurse was in the process of injecting my IV when I got shocked, so I felt the full voltage with zero medication. It was pretty bad, the equipment around me had not been pulled away so I was slammed into a monitoring unit about six inches above my head and chest. When it was over the first thing I asked was if I still had all my teeth. I can't say its like anything I've ever felt. Apparently the technician was new, when it was over Marchlinski gave him a serious dressing-down.
So the upshot of this EP study: All the remaining origin sites of the VT were on the outside of the heart, so no ablation was done. Dr. Marchlinski wants to go in from the outside, performing an epicardial ablation, a proceedure that has only been done since 2000. It requires going in under the sternum, and is a full day of surgery with full anesthesia. Dr. Marchlinski is confident about the location of the site, it is far away from areas of the heart that might be damaged by epicardial ablation, and he thinks it will be successful. He claims a 100% success rate for all epicardial ablations he himself has performed (the national average I looked up is 84%)
After that he is definitely recommending the ICD, as with all V-tach patients there is no guarantee that there won't be a relapse. One thing we learned with the accidental defibrillation is that I won't go into the continuous loop of shock-V-tach-shock that Dr Marchlinski was worried about before.
So my return visit is now scheduled for the week of the 2nd of June. With both the surgery and ICD it will be 4 to 5 days in the
hospital, and wil likely require more recovery than my past visits. The ICD limits activity for the 6 weeks post-implantation in
order to allow the leads to "bed-in" to the heart.