Science & Shepard’s Pie

At this point, I still have many questions regarding my diagnosis how it all adds up to the relatively morbid diagnosis of ARVD. On one hand I’m much more accepting of ARVD than I was 8 months ago – so much so that I can even picture myself saying to someone, “Hello, my name is John, I have ARVD”. My wife knows I can be a bit of a fighter, and a stubborn one at that. After my visit to Johns Hopkins when I told her that I think I should acquiesce if only on the topic of surgically implanting an ICD in my chest she stormed out of the room. It occurred to me much after the door slammed that she did not disagree with my judgement, but rather was vexed that I folded my cards so readily and uncharacteristically. She’s the one who’s suppose to tell me “when to fold ’em” because I never do, at anything.

This cardiac anomaly has been simmering on either the back-burner or the front-burner of my life since 2011. If you dig back enough, this blog is not about health or hearts at all, it’s about joy in the simple act of turning the pedals not only through life, but though the amazing twists and turns around places, people, and circumstances that I never could have fictionalized. (It’s also about a science guy trying to do his best at faking writing because honestly I don’t even know if “fictionalized” is a word and the more I ponder the thought the more I think I should create a software program that prevents me from sounding so daft.)

One irrevocable certainty is that I’ll be a stubborn science guy until I’m finally vindicated of that curse which will likely occur when I’m placed in the ground. Until then, I need numbers, I need graphs, I need case studies, I need to cross reference my data with others. Medicine is science and therefore there is an exact answer that previously collected data supports or one we can calculate within a given accuracy.

In my recent trip to Baltimore, however, I receive the a diagnosis which is a Shepard’s Pie. A little science, a little guesswork, a little leftovers from the guy they diagnosed last week. My travels and experiences have opened my senses to so many exquisite delights, that the lukewarm dish they are presenting on the menu appears all the less appetizing.

The Science of Shepard's Pie

The Science of Shepard’s Pie

In full disclosure, they are not wrong. You do not get to see the worldwide expert personally for a medical conundrum unless you are pretty screwed up. And as it turns out, as much as I’d like to prove otherwise and no matter how many KOM’s on Strava I can post in protest, the science says I’m pretty screwed up. Screwed up people need to take all the help they can get. So here I am a scientific misfit who’s hungry and cold Shepard’s Pie is on the table. It’s good, right?

It’s still too early to tell and too many results still pending to build a case but the more I write the more people reach out – also wondering if they have ARVD – and I continue to search to find information on ARVD. I find medical journals are quite helpful and I am extremely thankful for all the case studies conducted (and a special thanks to my statistics professor from a dozen years ago in helping make sense of it all) but really what I want to know is real people, with real stories, with real numbers, and real outcomes.

My final outcome is still to be determined. At this point the ARVD diagnosis is convincing enough to prevent me from cycling even though I feel the healthiest in years (and riding the best due to the lack of interruptive surgeries and arrhythmias). It’s also important to note that everyone has a different situation: a different family history, a different genetic sequence, a different history of fainting, a different EKG, a different cardiac MRI, etc, etc.

After getting this far I do know one scientific fact. Every person who is being tested or considered as having ARVD is wearing their heart on their sleeve.

A few numbers I have on hand at present (I’ll try to post more)
[Note: I’ll update this later since I’m missing a few stats considered in the TFC]
Negative: Family History of Cardiac Sudden Death or Heart Issues
Negative: History of fainting while exercising
Negative: Genetic test for the 5 desmosomal genes associated with ARVD
Negative: Cardiac MRI (conducted 3x, still negative for major or minor criteria)
Negative: Evidence of Fibrofatty Tissue in the heart, basically the definition of ARVD.
Negative: Signal Average ECG (looks good)
*Positive: (*Now: Negative) Arrhythmia (sustained) Corrected with Epicardial Ablation
*Positive: (*Now Negative) Inverted T-Wave in leads V1-V3. Although T-wave inversion is big flag for ARVD (50% of ARVD patients have V1-V3 T-Wave inversion vs 5% in the general population) this inversion can also occur very frequently in children, young women, and… you guessed it… athletes. The study that showed this also showed the T-Wave inversion reversed itself after a few weeks of rest – which is exactly what happened.
Positive: PVC rhythms emanating from multiple locations
Positive: Slightly dilated Right Ventricle
Negative: PVC Frequency: Measured 50-160 PVC’s per day. ARVD minimum is 750 per day, average of 4500-7500 per day, and can exceed 15000 per day.
????????: Newer 76 sequence genetic test, apparently can’t help with ARVD diagnosis but can help identify genetic links in the family (which or how many family members to contact if you are positive)

Height: 5’11” / 181cm
Weight: 156lbs / 70.7kg
Body Fat: 6.9%
VO2 Max: 74 ml/kg/min
Stress Test: 22 minutes (terminated by tester @ 176bpm since max effort was not requested)
PVC’s after stress test (recovery): none
LV end-diastolic diameter (4-chamber): 5.5 cm
RV end-diastolic diameter (4-chamber): 5.6 cm
Myocardial signal intensity: normal
Global RV dilation (mild/severe/absent): Mild
Segmental RV dilation (mild/severe/absent): mild basilar dilatation
Fat infiltration RV wall: absent
hypokinesia: absent
akinesia, dyskinesia, or dyssynchronous contractions/RV aneurysms: absent

Heart Pumping Skills @ rest
[Note: normal/average ranges in parenthesis]
ED volume: 217.27 ml (101 – 236 ml)
ED volume index: 114.50 ml/m2 (52 – 112 ml/m2)
ED volume/HT: 120.48 ml/m (60 – 130 ml/m)
ES volume: 80.93 ml (28 – 93 ml)
ES volume index: 42.65 ml/m2
Stroke volume: 136.34 ml (66 – 150 ml)
Stroke volume index: 71.85 ml/m2
Cardiac output: 7.92 l/min
Cardiac output index: 4.17 l/(m2*min)
Ejection fraction: 62.75 % (55 – 74 %)

ED volume: 279.47 ml (110 – 243 ml)
ED volume index: 147.28 ml/m2 (58 – 115 ml/m2)
ED volume/HT: 154.97 ml/m (65 – 134 ml/m)
ES volume: 173.41 ml (46 – 112 ml)
ES volume index: 91.39 ml/m2
Stroke volume: 106.05 ml (60 – 136 ml)
Stroke volume index: 55.89 ml/m2
Cardiac output: 6.16 l/min
Cardiac output index: 3.25 l/(m2*min)
Ejection fraction: 37.95 % (47 – 63 %)

Overall heart report:
Mild global right ventricular dilatation and mild right ventricular
ejection fraction reduction at 38%. No regional right ventricular wall
motion abnormality. No major or minor MRI criteria for the diagnosis of
arrhythmogenic right ventricular dysplasia (ARVD).

Doctor’s orders:
No activities to raise heart rate for the indefinite future [including table tennis]

… it still remains unclear exactly where heated board games such as ‘Battleship’ or ‘Monopoly’ fall into the prognosis. Watch this space for updates.

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3 Responses to Science & Shepard’s Pie

  1. Shawn Rosenthal says:

    Hi bud, you’ve been through a lot. Glad you’re posting all this to share. I’d really like to hear how you’re doing post epi-ablation.

  2. Craig Cook says:

    Hi Wayward,
    Realizing suddenly that I don’t know your name, despite reading most of your heart related posts. First, my condolences for your grandmother, she must have been awesome. All your most recent posts have been so compelling and well-written, thanks for sharing. Maybe I’m particularly sensitive because of the similarities in our stories, but truly, your blog is emotional reading.
    Now to my unsolicited advice: don’t give up! Definitely get an ICD, make sure they set it to shock nice and high so you don’t have any unnecessary zaps, definitely get it placed sub-pectorally so you can feel comfortable being active. Definitely join the yahoo ARVD group mmettera so you can get the full picture of everyone’s different experiences. Definitely get genetic testing done. And, if it comes back negative, as I suspect it will, definitely get back on the bike.
    I have some issues with the Hopkins approach that I’d be happy to share in a private email. They are a great resource, but I feel they are overly conservative. When you join the yahoo group you will see that everyone progresses differently, and those who do not have the currently identified markers simply progress less or not at all. There are a number of former athletes on the group who continue to remain active to different degrees.
    Anyway, I would love to continue this conversation, but I should be studying for a licensing exam I’m taking Monday. Best of luck and let us all know what your current status is and what you decide.
    Cheers, Craig

  3. oreoowner says:

    I probably missed this somewhere, but have you been genetically tested? I never even thought of it and no one suggested it until my local cardio referred me to the leading researcher and genetic cardiologist from italy (who happens to work in NY too-SCORE!). I also looked OVER and OVER data and tried to find people. Unfortunately, there are many people with dilated cardiomyopathy, but not with the genetic form I have which is different than most people.I did however find a few, and they seem to be doing well despite the many scholarly articles and journals online about the poor prognosis. Many things online are outdated and it’s amazing the different things doctors are working on (stem cell, artificial hearts, changing genes).

    I brought up the point of how many articles point out my diagnosis is so much worse than others without the genetic disease I have-my doctor told me not to worry, it could be decades before it deteriorates and the medicine they have now helps. He basically said each individual is different and no one knows as each disease is different, so to live my life. I was assured by the other doctors I can have a happy long life. Great news from doctor, not so great news when looking up things online. So I’ve decided to stop and take this rollercoaster called life. I armed myself with the best docs I can find and had an icd installed in September.

    My doc specifically does many people’s icds not only to function great, but also look great. I don’t even know it’s there most of the time, except my scar healed outwardly, so the scar is bulged, but not the chest. Either way, it’s there to save our lives just in case, so it’s a blessing we have it (even though we wish we didn’t have these diseases). Many information is found online, but remember everyone’s case is different. Some good sites to find other people in similar cases/situations I’ve found is the ICD support group online and . Facebook has plenty of good groups too (living with an icd, young hearts with icds).Good to hear your story-keep your head up!

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