Living, riding (and sometimes racing) with Exercise-Induced Arrhythmogenic Right Ventricular Cardiomyopathy
Tuesday, April 29, 2008
Treadmill Redux
The day after ablation and all is well in the world. Except that Marchlinski has ordered me back on the treadmill. It seems a bit soon after seven hours of cardiac cath., 24 hours with no food or water, and a fitful, uncomfortable sleep. But Marchlinski can do no wrong so I oblige. Afterall he is excited to test his work, and I am excited to prove myself fixed.
The treadmill looms large, This time I fear it. The resistance is ramped up. We get to eight minutes -the point at which everything came crashing down the last time. I cruise right by. The heart rate hits 150 without trauma, I do 12 minutes, the doctor in charge asks me if I want to continue. Feeling out of the woods and anxious to prove it, I say yes. We plod on. At 14 minutes I am at 163. There are PVC's on the EKG readout but nothing terrible. The doctor says it's my call, whenever I want to stop. Just because it is so good to be in control of myself once again I tell him I'll go to 15 minutes. At 15 minutes they shut the infernal thing down. I've reached 172 bpm with no V-tach.
Marchlinski comes by for a consult. He's highly pleased. But there are two caveats. First: because of the speed of my V-tach, and its ease of trigger with adrenaline, he is not recommending an implanted defibrillator until he knows more. He wants me to go home, resume life for three weeks, and come back for another electophysiology catheter study. In the meantime it is Beta-blockers and no exercise.
The other caveat is that I might not be fixed. There are areas on the outside of the heart which may not have been properly ablated but we won't know until things rest up and heal. So the return visit will determine that status. I'm so happy I did so well on the treadmill I can't imagine that I'm not fixed. I make a mental note to start some light exercise as soon as I feel up to it.
Sunday, April 27, 2008
I Wanna Be Ablated
The day after my treadmill blowout I was prepped, punctured and probed in a procedure known as catheter radio-frequency ablation. At midnight the night before I was not allowed to eat or drink anything until after the procedure. The whole thing seems pretty innocent, and if you peruse the 'net you'll find all manner of happy ablation explanations and stories. I've even read "geting (sic) wisdom teeth pulled is harder". My ablation, however, was epic.
There is probably a vested interest in making the procedure seem benign, hundreds of thousands are performed for all manner of cardiac anomalies, not just ventricular tachycardia. And it is a genuinely advanced, remarkable and relatively less invasive procedure, relative to, say, open heart surgery.
The electrophysiology lab is basically a specialized operating room. There were at least 15 large, flat screened monitors, including a bank of four perched on an arm above the operating table. The CPU power rivals NASA mission control. I'm wheeled in to the sound of classic rock as three technicians and nurses prep me. This involves entry-point hair removal, a gazzilion leads and wires stuck to my front and back (including a defibrillator), and the application of several lovely warm blankets which unfortunately cool down way too quickly. The room is cold, in the low 60s, and little naked me got to shivering almost immediately on the operating table. Prep took a good hour, then sedation was administered and I was vaguely aware of some fiddling in my nethers followed by a sensation of strange worming activity in my chest as the catheters went in. Doctor Marchlinski came into the room and the classic rock changed to easy listening. I drifted off to Sade's Smooth Operator. When I awoke my heart was being run through the gears, electrically stimulated in order to locate the sites of my V-tach. The heart is "mapped" via this process. V-tach is induced and the errant tissue located. It is bizarre, you have no control over your body, your heart is riding up Alp d'Huez but the rest of you is stock still. Full consciousness is required to get a proper cardiac response, so once they started mapping and ablating I wasn't allowed any more drugs. Once the mapping is done the ablation is performed with another catheter, tipped with what I imagine to be ET's finger.
My sites were large and varied. It took 55 "burns", individual ablations, to finish me up. The whole thing lasted 7 hours. Some ablations were without any pain. others felt like getting stabbed from the inside. Early on Dr. Marchlinski discovered that I had a large site on the exterior of my heart. He tried reaching it from the interior by zapping the bejesus out of that spot. That is what hurt the most. I begged for some sedation and got a little reprieve. By this time my back had gone numb from lying there for so long and I was shivering with cold. When it was over I couldn't move for six hours while the entry-point for the catheter closed up. The whole procedure, coupled with the lack of ability to move and thus being unable to pee for a total of 13 hours was, to date, the most uncomfortable thing I have been through. I don't care what the CIA says, there is something called a "stress position", and it can be simply being strapped to a bed for 13 hours.
Friday, April 25, 2008
So THAT's what's wrong...
Monday the 21st of April. University of Pennsylvania Hospital. I brought my running shoes, ready to pound the treadmll into submission. Now, bear in mind I haven't done anything other than climb some stairs in over one month. Upon check in I am on a perpetual cardiac monitor. Every move I make, my heart's reaction is recorded. Happily I find my low heart rate hasn't gone the way of my fitness. I'm getting gurneyed around the joint, all the while beating steadily in the mid-40's.
I step up to the treadmill. We go through the paces. There is a casually dressed intern, and a nurse, and we are talking bike racing -there always seems to be someone interested in bike racing. The doctor increases the resistance on the treadmill, I respond. I look over at the monitor and I am PVCing all over the place. Steady "sinus rhythm", the proper healthy heart beat, is muscled aside by wild, baroque lunges of the EKG's readout. I feel fine, though and my heart rate is now 120. The resistance goes up again, we are eight minutes into the test, and I'm starting to feel funny. I know from past VOMax testing that I shouldn't be happy with lasting anything shorter than 17-18 minutes. I press on. At 140bpm the EKG readout looks like the trace of a conductor's baton during a Wagner opera. I am in V-tach. My heart is thumping on my ribs at 300bpm trying to get out. I go pale and limp and they pull me off the treadmill and onto a table. There is a long period where they run around getting the defibrillator going. I start to get that receeding feeling, like the whole scene is moving away from me down a rabbit hole. Somewhere in the back of my mind I remember what my girfriend's brother told me. A trained paramedic, he knew of cases where a person can defibrillate (cardiovert is actually a better term) themselves by coughing hard or thumping their chest. As the doctor wheels in the defibrillator I give a hard cough. Five seconds later I resume sinus rhythm. There is a collective sigh of relief. I was in a 250 to 300bpm run of V-tach for over one minute. Now I know what this this is all about.
The V-tach was utterly wierd. It put alot of things in perspective. I realized that at least one of my previous episodes of passing out - three years earlier while riding my bike in France- was a run of V-tach. It felt just like that. But what I didn't understand was why this time, with just eight minutes of mild effort, it came so quickly and so strongly, after having no serious sustained manifestations for so long. Part of me feels that somehow being super fit and diligent about exercise actually helped supress the uglier symptoms of my condition. After all, why, after over one month of totally sedentary living, do I now have an extreme bout of V-tach? There is nothing that I have come across in my research that agrees with this, it is just a speculation.
There is also the possibility that I had myself looked at JUST IN TIME. If I had waited any longer, ignored it and tried to race, I'd be lying in some gutter on the side of the road dead of cardiac arrest.
The treadmill episode dramatically vindicated Dr. Marchlinski's opinion. It's strength and length suprised even him. From then on there were no more doubts.
Monday, April 14, 2008
Precedence and Prescience
Stive Vermaut, a victim of V-tach. photo ©: Daniel Schamps
Turns out that relative to the general population, there are an uncanny amount of endurance athletes with arrhrythmias, and dangerous right ventricular ones at that. A google search will bring you to myriad studies. Basically, I am almost a textbook case.
Among more well-known cyclists, there are two recent cases, one a happy ending, one not. Tour de France star Bobby Julich, whose particular arrhythmia was actually supraventricular, was cured with radio-frequency ablation. Bobby is now back at the top of his game. Stive Vermaut was not so lucky. Stive quit the sport after his first bouts of V-tach, was cleared to ride again, had another episode, quit for good, and died two years later of cardiac arrest. What isn't clear to me is this: what was the state of his treatment and why did he not get an implantable defibrillator (ICD).
Dr. Marchlinski gave me a three-pronged treatment strategy: radio-frequency ablation, an implantable defibrillator, and a lifetime on low levels of Beta-blockers. Initially it seemed heavy-handed. The only data he had to go on was my copious PVC's and a suspicious series of short runs of ventricular tachycardia during the ride I did with the Holter monitor. There was my previous history of fainting (two times) in odd circumstances, but that ws years ago, and to my mind it didn't all add up.
Nor did it add up to Dr. Larry Chinitz, my second opinion. He was suprised at Marchlinski's aggressive approach and thought further testing should be done. I submited to a cardiac MRI, and another echocardiogram.
Neither test proved any more, or less, conclusive. My mother -a licensed nurse who runs a floor at NYU medical center - was perhaps the most doubtful of all my "consultants". I decided to approach Dr. Marchlinski's treatment one step at a time. I had stopped all exercise, started on the betablockers, and waited for my next test - a treadmill stress test that, I was certain, would clear the air. Despite having been "out of training" for almost a month I was confident in my athleticism, and certain I would ace their little treadmill test.
Somewhere in the back and forth between NYU and the University of Pennsylvania, Dr. Chinitz and Dr. Marchlinski's opinions converged. I still don't know what did it, but Chinitz let me know that he was now in the Marchlinski camp of aggressive action. I shrugged my shoulders and booked my flight.
Tuesday, April 8, 2008
Mount Doom
Going backward to go forward. March 1 2008, and I'm standing on the starting line at the Rutgers University season opener for the Eastern Collegiate Cycling Conference. I am in the waning weeks of my thirty-eighth year and just having a number pinned on is an indulgence. I'm a graduate student at the Princeton University School of Architecture; I've spent the bulk of adulthood's first two decades as a slacker itinerant bike racer in Europe and the United States, and I really should be in studio building models.
Four years ago I ostensibly gave up the bike to resume my education and "get a real job", but I stayed in touch with the sport. I coached a team of young riders, using that as an excuse to ride myself. And I occasionally moonlight as a journalist, using that as an excuse for staying a sports fan. And from time to time I sneak some racing when no one is looking. Now, in the Collegiate Conference I race with guys who were busy being born the same year I was busy dropping out of college.
What I know now is that the Rutgers weekend will probably go down as my last bike race ever. As I struggled to keep up in a field that I would have easily been competitive in just a year before, I had no idea that I was flirting with something akin to death. I finished the race weekend inordinately fatigued, and in the following days post-race recuperation was proving hard to come by. My pulse, low and occasionally irregular -as is common with endurance athletes- was all over the map. I sat in a lecture feeling as if my heart was doing pirouettes in my chest. I went down to university health services. They put me on an EKG post-haste. I was having what is known as Premature Ventricular Contractions (PVC)s, a common phenomenon that many people experience. The trouble was that mine were wierd looking, came in extended runs, and had been going on for some time. I was sent to a cardiologist that same day. He was intrigued but not concerned. An echocardiogram gave no further clues. My heart was structurally solid, "an athletes heart" I was told. I expected nothing less.
I was given a Holter monitor to wear for 24hrs. This is a continuous EKG that records all your hearts activities. I was advised to do everything I normally do. So that day I went out for a two hour ride. I hit some of the local hills, making sure I got up into the levels of exertion I would normally reach in a race. I felt odd, like I was bottoming out on the hills, but I seemed to recover normally from the efforts. The next few days I was too busy to ride, which was fortunate because I was feeling better and beginning to think that the whole heart arrhythmia thing was silly. Then I got the call. Stop what you are doing, and go see a certain Dr. Francis Marchlinski at the University of Pennsylvania. My Holter monitor readout was grim.
Dr Marchlinski is a guru in the world of cardiac arrhythmia. Much in demand, the only reason Marchlinski deigned to take my case was because it was interesting to him. At the time I didn't know this and was feeling pretty flippant about the whole thing. I had already missed two days of school, which is alot in the fast-paced curriculum of an architecture masters program. I took another day off and headed down to the University of Pennsylvania hospital. After another EKG and a 45-minute inquisition by an intern, Marchlinski himself appeared. Tall and rangy, he is a wizard-like man in his mid-50s. At once kindly and intense, he seems to get lost in his words as he stares off at some Mount Doom no one else can see. After a few minutes with him one is suprised by the lack of a beard, robe and staff. Marchlinski's assessment was this: I have a serious cardiac anomally that could kill me at any time. Ventricular tachycardia, V-tach. Mount Doom.
There are myriad forms of cardiac arrhythmias, some are dangerous some are not. Simply put, certain arrhythmias are caused by scar tissue that develops in the heart muscle that create electrical signals that override the heart's normal rhythm. The location of the sites is the determining factor in assessing the malignancy of the arrythmia. Ventricular Tachycardia is one of the most dangerous and unpredictable of arrhythmias. Because it originates in the chambers of the heart most critical for sending blood to brain and body V-tach can shut a body down and do irrepairable harm. When treated, there is never absolute certainty that V-tach won't come back.
I left Philidelphia perplexed, bemused and disbelieving. I've always known my heart was a bit wacky, but it was hard to imagine being as healthy and athletic as I was and being so close to a, um, terminal moment. I spent the next week doing research, calling medically inclined friends and relatives. Next... what I found.
Subscribe to:
Posts (Atom)