Normally abnormal.

Athlete's Heart vs. Cardiomyopathy, from http://impruvism.com/athletes-heart-1/

The image above is about my heart, ARVC and the results of a recent round of testing, including an MRI. But let's skip to the part where I get an MRI with metal hardware in my chest that basically controls my destiny. This has got to be lowest-common-denominator interesting because MRI techies would have you believe that any metal you happen to absent-minded meander with into an MRI tube  is going to be ripped off - if not through - your body in some cinematic manner. Apparently this is not the case.

"It's not really about the MRI pulling things around in your body" the electrophysiology fellow who was prepping me for my test told me..."its more about the heat."

This was not the answer I was expecting, nor was it reassuring.

"We try to keep the ICD from heating up inside of you."

So, the magnetic field of the MRI heats metal. Those of you with an ICD know that the unit functions with "leads" - metal wires that are embedded in the heart that detect heart rhythm and deliver pacing, and eventually the shock, should it be needed. One can imagine an unpleasant scenario the medical journals refer to as"radiofrequency-related myocardial thermal damage ".

This did not happen to me. The MRI went according to the book. The tube was claustrophobic, I was nervous, but it all worked out. I prefer to keep my eyes shut once the donut has swallowed me. Knowing I have no ability to move is not such a big deal when I can't see the closeness of the machine. 

After the first half-hour or so I started to enjoy myself. There is a hell of a lot of breath-holding with a cardiac MRI. Apparently this is key to getting good images. I was an easy patient and my case was almost boring, as evidenced by the EP fellow playing Angry Birds as I was wheeled through the control room when it was over.

The results, I would learn later, were pretty encouraging. As a reminder, the MRI was performed to determine if my bout with mycarditis a few months earlier had any lasting effects on my heart. It was also done to see if there had been any degradation to my heart, 5 years after my previous ablations and ICD implant. ARVC is a "progressive" disease that is thought to be negatively affected by exercise. By my estimation I've ridden my bike around 30,000 miles since recovering from my ablations in 2008. In 2009, determined to see if I could get back to a prior level of fitness, I did several bike races on my long-disused "Category 2" USAC license. Since then I've been riding as much as I wish as fast as I wish. Which is not particularly much or fast - but still mostly in the 75 to 95% of max heart-rate zones.
All highly ill-advised by the ARVC specialists.

My MRI showed that my heart was unchanged since my first MRI in 2008. In fact, one particular metric had improved - my Right Ventricle End Diastolic Volume had reduced slightly. This is important because right ventricle enlargement is a major symptom of ARVC, though it was by no means showing that my condition was reversing, either.

The end result was that I am exactly where I was in 2008, heart health and fitness-wise. My heart is a bit more enlarged than a typical athlete's heart, but no more so than before. If ARVC is a progressive disease, I am not progressing. More about what this may or may not mean in a future post...

Grim Reaper Euro-Ambulance

Sooo...did I mention I'm not the most diligent patient? After my run in with the Unknown Bugs I was thoroughly chastised by my heart doc Frank Marchlinski, various members of his support staff, my mother, and my Person (Molly), for not having seen a doctor or sent in my ICD data for the better part of two years. I was healthy, what can I say?
But I vowed to be more On The Ball; and that meant taking Marchlinski up on his offer of a repeat MRI and PET scan. The MRI was to judge the condition of my heart 5 years after my initial treatment, the PET scan was to determine if there were any immediate effects from the suspected myocarditis.
Now, those of you with an ICD might be saying to yourselves "but, I was told you can't do an MRI with an ICD..." And you would be sort of right. As it was explained to me, very few hospitals will do an MRI on a patient with an ICD, but in fact it can be done. U Penn is one. Specially trained technicians are brought in, the machine is recalibrated, an electrophysiologist and ICD specialist stays in the lab to monitor things, and it takes forever.
But by the time I was fully recovered from my sickness I had better things to do than truck up to Philadelphia and become the filling in various high tech donuts for the better part of a day. Or so I thought. 2012 became 2013 and I still hadn't made my appointment.
It was some time in January that Molly and I found ourselves woken every morning at a quarter of seven by our neighbor's alarm clock. This was not particularly notable, apart from the fact that it sounded like a distant Euro-style ambulance (eee-naa!...eee-naa!), and it seemed to be a reminder of immanent doom. As if all my years of damaging myself in the bike racing battlefields of France had come to this. If I didn't make the damn call and get the MRI, the grim reaper was closing in behind the wheel of a bubble-topped red Mercedes "Sprinter" with "Secours!" written on the side. Over a month went by with this constant early morning reminder niggling me to make the bloody appointment. Then, thankfully, we moved.
Excitedly putting together our new home, I forgot about the MRI. Then, quite literally the morning after our move, the grim reaper Euro-ambulance woke us up again with its incessant "eeee-naaa." We looked at each other incredulously. What were the chances that our new neighbor in our new apartment had the same alarm clock as the neighbor in our old apartment? I pondered this in the shower a few mornings later, up earlier than usual, when the alarm went off again. Strangely it seemed just as far away as it did in bed, by which I mean, basically right next to me. It was then that I realized, the grim reaper Euro-ambulance was my goddamn defibrillator.
I had my ICD interrogated by a local EP. The battery was almost dead, and it had been for quite some time. I couldn't actually say when I first started hearing the alarm, but it was well over a month. I needed a new battery post-haste. This was very premature - ICDs are supposed to last at least 8 years, and mine was in its death throes at barely 5 years-old.
This was actually a relief to the crew at U Penn. I could get my MRI immediately before my ICD replacement surgery. This meant that if the ICD should get fried during the MRI it really didn't matter, I'd be wheeled upstairs for a new model directly. Knowing the grim reaper was empowering: I made my reservation, one day for the PET scan, one day for the MRI and ICD replacement.

Next episode: New battery...same heart..

Sidebar: Emma Carney's EI-ARVC

Emma Carney is a former world champion triathlete. You can read about her palmares here. Most online explanations of her career-ending heart condition don't go into very specific detail about her "clinical presentation". (i.e the Wikipedia entry that says her ICD is implanted in her right ventricle)
Anyway, what is striking about Emma's case is that it spawned the first serious, peer-reviewed, medical journal article presenting the idea of an Exercise Induced ARVC that is apart from the conventional form of ARVC. This article was published in 2009 in the journal Heart, Lung and Circulation. Here is  the introduction:

Recurrent sustained exercise induced ventricular tachycardia (VT) in elite ultra-endurance athletes is a rare but serious problem. Recent studies indicate that most serious ventricular arrhythmias (VA) in elite endurance athletes arise from the right ventricle (RV).   In some the diagnosis may be arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). In others it is postulated that repeated extreme exercise may damage the RV thus providing a substrate for arrhythmias.  In such cases the term EIRVD/C is applicable.   Distinction between these two entities (ARVD/C and EIRVD/C) is important. ARVD/C is an inherited disorder characterised by progressive fibrofatty replacement of RV myocardium eventually leading to ventricular arrhythmias and right heart failure.   The underlying pathophysiology is a genetic abnormality of cell adhesion proteins such as plakoglobulin, desmoplakin, plakophillin-2, and desmoglein-2.    In patients with ARVD/C exercise may precipitate ventricular arrhythmias and aggravate or accelerate the condition but it is not the fundamental cause of the disorder. On the other hand EIRVD/Cis thought to be the cumulative result of repeated bouts of extreme endurance exercise each of which has caused a small amount of damage to the RV.   We report a case of exercise-precipitated recurrent sustained monomorphic ventricular tachycardia (VT) occurring in a 32-year-old multiple world champion female triathlete without a family history to suggest ARVD/C and in whom investigations support a diagnosis of EIRVD/C rather than ARVD/C.

The case report portion of the article goes on to explain her ARVD/C diagnostic criteria  - which are exactly the same as mine...apart from the bit about world status in triathlon...

An ECG showed sustained monomorphic VT with LBBB morphology. DC cardioversion was required. She subsequently suffered further similar episodes each related to exercise. A typical baseline ECG showed sinus bradycardia with anterior T wave changes. Echocardiography showed dilated ventricles with normal left ventricular systolic function but moderately reduced RV systolic function. An MRI scan  confirmed the above findings and showed no features to suggest ARVD/C. Coronary angiography was normal. An RV biopsy showed fibre hypertrophy with evidence of patchy fibrosis but no evidence of fatty infiltration... The absence of a family history and lack of typical changes on the MRI scan and cardiac biopsy make a diagnosis of ARVD/C unlikely in this case. Furthermore it is improbable that someone with ARVD/C could achieve world status in triathlon over such a long period of time with this diagnosis. We believe it more likely that the athlete suffers from the newly described condition EIRVD/C." 

Next episode: my heart - 5 years and 30,000 miles after epicardial ablations and ICD...

Attack of the Unknown Bugs

Happiness is a quiet heart. From my last update at the close of 2011, to October 2012 things were entirely quiet. The rare PVC and rarer 2 or 3 seconds non-sustained ventricular tachycardia was about all the noise it could muster. Over this period I rode 5000 odd miles, did a trek in Nepal, and generally lived as actively as ever. Then some strangeness occurred. Probably I should have dusted off the old blog and given you the blow-by-blow, but I didn’t, so here’s the summary.

Early October, 2012, I get really sick one Friday night. Fever, chills, aches, but the worst of it is chest pain. The chest pain comes in waves, and subsides a bit if I lie flat. The fever is highish, 102 or so. By late Saturday I head to the emergency room. Unfortunately I choose a hospital by virtue of its reputation for having a quiet emergency room, not by its reputation as a hospital. After getting my vitals and the Reader’s Digest version of my health history they do an echocardiogram and blood work. The echo shows a depressed ejection fraction, 45% if I recall. The blood work shows elevated levels of troponin,  an enzyme that indicates the heart is not happy. The ER doc suggests I am suffering from myocarditis or periocarditis. I am admitted for observation.
Myocarditis is an inflammation of the heart, usually brought on by a bacterial or viral infection, periocarditis is an inflammation of the sac that lines the heart. Both would be responsible for the acute chest pain I was having, the fever, etc. 

My fever persists, antibiotics are tried. Further tests show that I do not have any known influenza, and the antibiotics are not working, so a bacterial infection seems unlikely. Unfortunately the hospital has no cardiologist on call on the day I am admitted, and the next day an ancient man who calls himself a cardiologist is assigned to my case and immediately asks me why I am not on ACE inhibitors, and beta-blockers, given my history. This sends up immediate alarms in my mind. I have been successfully steering clear of drugs since after my ultimately successful ablations in 2008. By Monday morning, with the chest pain gone and  the fever subsiding, and none of the three doctors who came to see me (cardiologist, internist, infectious disease specialist) is able to tell me what is wrong apart from some notion that I should be better medicated. I insist on discharge.

I schedule a visit with my trusted EP at U Penn, Dr. Marchlinski, for the following week. Seven days after the initial sickness my troponin levels are normal again, and my EF is up to 50%. Marchlinski cannot say with certainty what it was, but suspects a non-influenza virus that decided to snack on my heart. In fact, this suggests what we have suspected all along, that my heart is unusually susceptable to attack, and that something like this may be responsible for the original scarring that caused my condition. He wants me to do no exercise until I am completely recovered, and to come back eventually for an MRI and a PET scan to determine if there is any new scarring. 

It takes me over six weeks to recover fully. The second week out the fever returned, along with an incessant cough. I miss two weeks of work, visit local GP  who can't shed any light on my situation either and doesn't want to start up antibiotics again. Finally by the second week November I'm starting to feel well enough to do a little exercise, though the cough doesn't abate until Christmas. 

Out of the woods finally, I start to think about scheduling that PET scan and MRI... 

Jumper cables, anyone?

After two years of silence, I'm kicking this spaceship back in to action. There are two reasons for this, the first, is I had a bunch of harrowing heart adventures over the past year that I probably should have been sharing. The second is that somewhere in New England there is a guy going through almost exactly the same series of events I went through when my heart began acting up 5+ years ago, and he's blogging about it and it is worth the read. So, if anyone still tunes into this blog, mouse on over to the Wayward Cyclist while I pull together my adventures of the past year and all the fun new things I've learned...