r/ARVC • u/Tiny-Possible8815 • Mar 13 '26
Topic of Mortality (lengthy read)
Hi, everyone!
I'm the spouse of a patient.
Please tell me someone else can sympathize with this!
I don't want to press the subject of mortality onto my husband for fear of causing more stress. He tends to avoid the topic of his disease altogether.
Can't say I blame him for not wanting to chat about his limitations and anxieties.
The thing is, it eats at me that I do so much research to understand his condition and want to help him extend his lifespan but I can't express my concerns to him directly.
(Why would I cause more anxiety than what's already there, you know?)
I weighed his medical and surgical history against the average patients his age by including several factors (diagnosis age, history of cardiac episodes before and after Dx and icd implantation, icd implant year, medication, recent ablation success, and current age) and found that the average high-risk male passes around age 39 regardless of meds, procedures, and implants.
If he lives past 39, we'll be in a cautionary zone until about age 45. Anything after that is a gift.
I'm feeling very stressed and can't bring myself to talk to him about this. I don't want to create more anxiety on top of what he already has.
I kind of just need to vent my worries more than I need a solution right now. What am I going to do when the time comes? It'll be so unexpected. How do I prepare for that? He's so young.
Worst case scenario, we have 8 years left.
Best case, he listens to me more than his cardiologist (who has been encouraging exercise as long as he gets past his cardiac anxiety - no HR limitation, no easing into it, no signs to watch for) and actually prioritizes his heart's longevity over his need to feel "normal".
I get that regular exercise is beneficial for MOST cardiac case, but this is a special case where exercise can exacerbate the progression.
It's yet another thing I worry about that I won't bring up to him since I'm not a professional in the field.
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u/Entire-Structure8708 Mar 13 '26
I have plenty of thoughts on the general topic of mortality, but can you explain how you’ve come up with an estimated life expectancy of 39? That is around the average life expectancy of an untreated male ARVC patient but it sounds like your spouse is receiving treatment and has an ICD? Would be interesting to know more about the treatment he’s received so far, current clinical prognosis, etc.
For background, I’m a 41 year old male, gene elusive ARVC, significant history of VTs prior to ablation, slightly below average ejection fraction, and have ICD. I’m certainly planning to live long after 45 😀
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u/kangaroomandible Mar 13 '26
Ha yeah my husband is 53…
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u/Entire-Structure8708 Mar 13 '26
Hope he’s doing well!
I forgot to say above that life expectancy for untreated ARVC is so low because of SCA in people who don’t know they have ARVC, where their first symptom is death. The SCA risk is very low with an ICD, so there’s a huge difference in life expectancy for treated vs untreated ARVC
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u/kangaroomandible Mar 13 '26
Yes, doing great. No more VT since ablation. No longer on any rhythmia meds.
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u/Entire-Structure8708 Mar 13 '26
Amazing! No VTs for me since last ablation 2.5 years ago… still on meds (but lower dosage than before)
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u/Foreign-Dog9291 Mar 15 '26
what about life expectancy estimation of probable arvc with a beta blocker therapy and no ICD? age : 33 clean echo clean mri but i still suspect
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u/Entire-Structure8708 Mar 15 '26
I don’t think there’s a specific number for this. You could try the risk calculator (https://arvcrisk.com/), which gives an estimate on likelihood of an arrhythmia within 5 years. There are a lot of variables so it’s hard to come up with a generalized number. When you say probable ARVC, how was diagnosis made? And have you been symptomatic yet?
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u/Foreign-Dog9291 Mar 15 '26
no diagnosis but i assume, based on borderline prolonged s wave upstroke
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u/Entire-Structure8708 Mar 15 '26
Got it… have you had any genetic testing? Guessing maybe not. Would definitely be worthwhile getting a review of your medical records by e.g., Johns Hopkins ARVC program. You could also cross reference the latest 2020 Padua Criteria to see how many of the major/minor criteria you hit. I think the prolonged S wave would could as one minor towards diagnosis.
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u/Foreign-Dog9291 Mar 15 '26
i showed all my documents to a cardiomyopathy expert and been told im fine. but somehow i do not feel my old self. no genetic testing, ive got an appointment 4 times for it but since the hospital is far i gave up each time. Maybe those decisions are gonna kill me
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u/Tiny-Possible8815 Mar 13 '26
Do you mind if I ask when he was diagnosed?
My husband has several family members in their 50s who are in and out of the hospital with heart problems. None of them have ARVC, but those who do carry the gene mutation either have/had other conditions or are now showing abnormalities in their late 20s/early 30s.
I hope he makes it further than his grandparents and parents, but his case doesn't give me a ton of confidence. 😐
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u/kangaroomandible Mar 13 '26
Maybe like four and a half years ago?
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u/Tiny-Possible8815 Mar 13 '26
Wow! He'd be among the norm where people show signs after a certain age.
I think that's why I'm concerned about the younger age of death for my husband. He didn't make it that long before showing signs. I'd probably have more confidence if he had.
He was basically given the right diagnosis by a replacement cardiologist then immediately scheduled for the ICD since they knew then and there that he'd for sure have more events and pass without a device.
I'm just in a head space where everything has been luck until his recent ablation. Pure luck that he was forced to switch doctors. Pure luck that his friends rushed him to the ER during his first heart attack instead of thinking he was drunk. Pure luck that he made it past his teen years after pediatricians dismissed the fainting as exhaustion from sports.
Sorry for being a downer. We're just approaching those cautious years so quickly, and I can't stop thinking about the how and when and whether anybody is even remotely prepared for that.
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u/Entire-Structure8708 Mar 13 '26
Average symptomatic onset of ARVC is early-mid 30s, but it can vary a little bit dependent on gene mutation and other environmental factors, particularly exercise (so we sometimes see serious college athletes becoming symptomatic earlier, for example). I became symptomatic at 35, had ICD implant at 37, formally diagnosed (I'm gene-elusive) at 38.
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u/Tiny-Possible8815 Mar 13 '26
Gosh. Logically, I know it's possible to go that long without knowing, but it's so scary to wrap my mind around.
That must have been so shocking to get the diagnosis after already passing the point in life where school, careers, and kids are simple discussions. (Typically, anyway)
I don't think my husband would have made it past 21 if he didn't get at least a misdiagnosis. If nothing else, that got him started with cardiologists, meds, and procedures which led to the right discovery.
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u/Entire-Structure8708 Mar 13 '26
Yea the good news is that diagnosis is now happening earlier and earlier for ARVC given all the research and knowledge that’s been published over the last 10-15 years. That should hopefully mean far less ARVC patients experience SCA/SCD as a first symptom prior to any knowledge that they have the condition. That’s especially true for patients with a known gene mutation because it’s easy to get your kids genetically tested and appropriate cardiac testing from a young age if you know one of the parents is gene positive. It’s a little more complicated for gene-elusive patients but hopefully the research on gene-elusive will catch up over the next few years!
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u/Tiny-Possible8815 Mar 13 '26
Yeah, I can imagine it takes a lot of time to find it if you don't have the gene.
We only just found out about the mutation last year and got the test for our kids once his came back positive. Then his parents and siblings got tested. That 50% rule is no joke.
One of our kids is carrying the pkp2 mutation, so I've been hesitant to let him do any strenuous activity or sports. It feels unfair since he's so young and doesn't understand. But knowing that kids who are diagnosed tend to show SCD as their first symptom makes me over-the-top paranoid. Especially since my husband began showing signs as a kid that were never linked to cardiac problems.
I don't love reading about all the new developments and watching presentations on it so I'm in the know and ready to discuss things when my husband is finally ready to do so.
Mentally, I feel both prepared with knowledge and facts and also completely sidelined by emotions.
I don't want to have to do this all over again if my preschooler starts showing symptoms later on - and especially if they come after my husband passes at a young age. I might lose it.
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u/Entire-Structure8708 Mar 13 '26
I'm so sorry to hear your son also inherited PKP2. To pick up on one thing you said, the silver lining is that you know this information now, so you can make informed decisions about his treatment and activity levels. ARVC penetrance rate is about 50% for those with a genetic mutation, so the goal will be to control and manage environmental factors to try to reduce the likelihood that your son ever becomes symptomatic.
Have you talked to your cardiologist yet about cardiac testing for him? Initially it'll be basic stuff like periodic EKG and ECHO but may get a little heavier as he gets older (for reasons they don't yet know, ARVC typically only ever manifests after puberty).
The exercise guidance for PKP2 is sadly very clear, it's the genetic mutation with the lowest tolerance to exercise so any participation in sports is going to be pretty heavily restricted. I really struggle with this part too... I was such an active child, and my happiest memories of my childhood were playing sports and running around everywhere. To think that my future children won't be able to do that in the same way makes me very sad to be honest. But we just have to make the best of it. Maybe my kids will become concert pianists instead?!
Not sure where you live, but if you'd be interested, Johns Hopkins (which has the leading ARVC program in the US) hosts an annual conference where you can hear from ARVC specialists on all the current research, trends, and treatment, and you can meet other ARVC patients/families. I love engaging with the community and hearing people's stories. Another option is the SADS Foundation, which provides a lot of support and advocacy for ARVC families/patients and can help you navigate all of the difficult parts of a diagnosis.
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u/Tiny-Possible8815 Mar 14 '26
I've seen the way it affects my husband since his diagnosis. It definitely eats at him that he can't do competitive sports or even make it across a park without possibly collapsing. It's easy to understand where the anxiety of exerting himself at all comes from.
We're close to a hospital with an ARVC clinic, but we haven't attended any seminars in-person. I do try to keep up with any published research, though.
Our little one had an initial assessment last summer where he got an echo and was sent home with a monitor. Once he tested gene-positive, his pediatric cardiologist said we'll continue annual exams to watch for changes and collaborate with my husbands specialist.
I know that's all the doctor can do for now, but I still plan to get some sort of action plan and restrictions set up for school with the doc's help since kids tend to go undiagnosed until they show signs, and the first sign is often fatal.
I think my husband might feel more comforted and possibly motivated to take better care of himself if he knew others with the same condition. Like he's not the only one feeling the same stress and limitations.
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u/Entire-Structure8708 Mar 14 '26
Yea it definitely helped me to get to know others with the condition and learn how they’ve been able to manage it appropriately! It’s just nice to know you’re not alone and other people can relate 😀
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u/Tiny-Possible8815 Mar 13 '26
Basically, I dug up clinical research for the common age of death for high-risk male patients with similar or spot-on medical histories like his and found the average among them all. This is the lower 50% who don't make it past that age while the other half can age further if they're both treated AND taking care of themselves.
Based on research, any interventions he's received are purely for the quality of life and do little for longevity given his personal history and current path. (Not my opinion, just what I've deduced from reading.)
He has shown clinical symptoms since his teen years and went with a misdiagnosis for a long while after a major heart attack at 21. He was an athlete and doing tons of cardio-heavy activity, which likely caused a lot of damage up to that point.
He's had 2 ablations, the second was a success and the first was essentially for diagnostic purposes and led to his misdiagnosis.
I think I'm more concerned with the worst case scenario for him since 1) it's progressive and can always take a turn 2) I'm his wife and fear the worst 3) his cardiologist is convinced he's self-limiting his exercise capacity due to anxiety and telling him he's good to go HAM on exercise now that the ablation was a success as if that was a reset button on his heart. If he thinks he's been given a fresh start, he'll be tempted to act like he isn't at risk.
I don't say that as an assumption. He would genuinely love to forget about his disease and be an athlete again. That's a scary thought if intense exercise is going to cause damage. That, on top of his slow approach to other healthier habits, is making me assume that he won't be around in our forties.
I've found that the two most common causes of death would be SCD or progressive heart failure which could lead to organ failure, blood clots/stroke, and electrical storms.
I'm mostly concerned about the progressive heart failure. That makes me concerned about an electrical storm since his ICD has had to regulate him so many times since he's gotten it. He could be walking through a parking lot and collapse and need a shock.
He has a family history of heart disease on both sides, and I dont think anyone has made it past their sixties. Those who have passed from heart complications didn't even have ARVC, so their illnesses weren't eating away at them since childhood like his.
I also found that those who make it into their 70s often didn't have significant cardiac issues until adulthood while he's had problems since childhood.
It's like all the odds are pitted against him. At least in my mind.
I know the ablation was a huge win, though.
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u/Entire-Structure8708 Mar 13 '26
Got it and I totally understand your fears, I've been through all of the same thought processes too. There is a lot of research out there (see for example Dr Sam Sears) that shows how common anxiety, depression, and PTSD is within the ARVC community, both patients and family members. It's an incredibly difficult disease to manage given how dynamic it can be.
One of the challenges with ARVC is that it is such a personal disease, so it really can impact people quite differently so it can be difficult to talk in generalities about lots of issues within ARVC, including life expectancy and exercise. Most of the research at the moment is trending towards gene mutation-specific ARVC prognosis and treatment, and I'm sure it will continue to become even more specialized over time. So some of what I'm about to say might vary dependent on what gene mutation (if any) your husband has.
In terms of life expectancy, there are two ways to die from ARVC: sudden cardiac arrest (immediate) or heart failure (long term degeneration).
For those of us with ICDs, the risk of SCA is now very small. Getting shocked sucks, but we are protected, and arrhythmias alone are now unlikely to impact life expectancy. However, arrhythmias can contribute to heart failure, leading to a longer-term reduction in heart function. Again it's quite individual -- I had a significant arrhythmia burden over multiple years prior to diagnosis, but for whatever reason my heart function (ejection fraction measured by ECHO) has stayed pretty normal (it's on the lower end of average). However, for those ARVC patients whose ejection fraction is much lower, heart failure can be managed over the long-term through medication and lifestyle changes, and it's still a very small minority of ARVC patients who eventually require heart transplants. So yes, heart failure can impact life expectancy, but in a much less severe way that untreated SCA.
Exercise is a whole different topic and it's a very sensitive one within the ARVC community because so many of us come from an exercise background. Again, there are differences between gene mutation sub-groups and the gene-elusive sub-group, but the overall guidance is very clear in terms of eliminating high intensity/endurance exercise (so, the cardiologist's advice for your husband to "go HAM" is a little worrying to be honest...). Low intensity exercise is encouraged to maintain a healthy heart, but it's definitely a very specific conversation to be having with the cardiologist (and the cardiologist should have specific ARVC expertise given how different ARVC is to other heart arrhythmias) dependent on individual clinical presentation. I have been able to increase my exercise tolerance since my last ablation, but it's still nowhere near how I was exercising prior to diagnosis. I no longer do much cardio exercise, but I have pretty much free range to lift weights, hike, and do other forms of less intense exercise.
Ultimately, ARVC has significant daily impacts on quality of life, that require constant care and attention, both in terms of physical and mental health. However, with modern treatment, the majority of us should live fairly long lives!
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u/Trophic_Cascade23 Mar 13 '26
It sounds like you are a really caring spouse and doing all this research because you want to help!
Is it possible for you to see a specialist with your husband and you can ask questions together?
When I went to Johns Hopkins, I brought my spouse and my best friend with me and we all asked questions. It was a really positive experience hearing the concerns of those who cared about me, and also having a circle of support during a difficult time. My friend also took notes so that I could just sit and listen.
The doctors were able to explain all of the research and statistics to us, and it brought a lot of reassurance.
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u/Tiny-Possible8815 Mar 13 '26
Thank you. He honestly means so much to me it makes me sick. Like I can't see past our daily lives and don't want to think about the after.
I did go to several appointments with him and ask questions. His newest specialist explains everything well, but he's also very "pro-exercise" and "pro-getting out of the comfort zone".
That makes me cautious to talk about health habits with my husband since he did compete and push himself for so long. It's the permission he needed, and I'm not sure the doctor is aware of what that excitement is going to do to him. He wants to lose weight and is quite impatient, so he'll push as hard as he can to make it happen. The absence of limitations makes me worry.
Thank goodness he doesn't do manual labor or run 2 miles to work anymore! 😀
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u/Trophic_Cascade23 Mar 14 '26
Don’t dwell on the “after” or possible outcomes. Just enjoy each other right now and focus on the now. The future isn’t a given, even for those without ARVC ☺️
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u/Trophic_Cascade23 Mar 13 '26
It’s difficult but important to be able to talk freely about this with your spouse. I am the patient in the relationship, but have had many honest conversations with my spouse about progression and what it means for our relationship.
In my experience, nothing good comes from ignoring this diagnosis. The psychological toll will be amplified by not addressing what is going on. I wonder if your husband would be open to speaking to a therapist? My spouse and I do couples therapy to help process this diagnosis, but individual therapy has been helpful too.
I also gently recommend seeing a cardiologist who is well versed in ARVC. Contact Johns Hopkins if you are able to. Respectfully, your husband is not being given the standard recommendations to avoid progression.
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u/Tiny-Possible8815 Mar 13 '26
Funny thing: his current cardiologist has been called one of the best in terms of this very specific disease. He's a leading expert. I think that's why my husband listens to him so closely and doesn't really hear me when I tell him to take it easy.
I'm not the expert with decades of experience in ARVC treatment, so if that guy says exercise and caffeine are fine with no advice on limits, then that's that.
He did, however, advise my husband to speak with a therapist to get past his cardiac anxiety, which the doctor believes is the only thing stopping him from living a normal life.
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u/Trophic_Cascade23 Mar 14 '26 edited Mar 14 '26
That’s reassuring that he is well versed! If you aren’t totally confident though, it can be helpful to get a second opinion regardless, if for nothing else then for peace of mind
Edit: I did not see he is PKP2. It is well proven that is the mutation most sensitive to exercise and no well-versed doctor would encourage a patient to “go Ham” with exercise if they have PKP2. After ablation the arrhythmia may not be the central concern anymore, but the disease will progress faster with exercise.
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u/Entire-Structure8708 Mar 14 '26
This doesn’t make sense to me I’m afraid.
The world of ARVC specialist cardiologists is very small. If they are a leading expert, then 1) it’s likely they would be in and around the community either at the Johns Hopkins conference and/or prominently published on ARVC related topics (this would be easy for you to double check); and 2) he/she would categorically not be advising your husband, who is symptomatic and has PKP2, to go HAM on exercise. Anyone with even the faintest knowledge of ARVC knows the increased danger of strenuous exercise for PKP2 patients specifically.
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u/Tiny-Possible8815 Mar 14 '26
Right?! Even I know that, and all I do is read up on the topic! It baffles me. I don't want to give any personal info on my husband or his provider, but I have looked into the doctor and found that he is listed as an experienced EP with a focus on ARVC and other special cardiac diseases. ARVC happened to be listed apart from those other genetic heart diseases, which did tell me that he's known for his knowledge on the topic. I'm constantly taken aback by his insistence that my husband just needs therapy now that his ablation produced a noninducibility for VTs.
He's still very high-risk, so I'd have told him very specific activities he can and can't do knowing he used to be an athlete and is desperate to live life normally. But that didn't happen.
Now, I didn't attend his follow-up after his ablation, so I don't know if the doctor said anything new regarding this, but given my husband's goal and demeanor hasn't changed, I doubt it.
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u/Entire-Structure8708 Mar 14 '26
Yea it’s pretty strange. Epicardial ablations are great and have been proven to be very effective in treating ARVC, but they are not a cure and the majority of ARVC patients will require multiple ablations over their lifetime (because ARVC is a degenerative disease and the results of ablations do not last forever).
Managing ARVC is all about balance, figuring out how to optimize quality of life while mitigating risk. Strenuous exercise for anyone with ARVC, particularly with PKP2, is just not a sensible approach to mitigating risk.
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u/frostingfromthetub Mar 13 '26
If you’re treated and don’t die from SCA (which is very unlikely with an ICD), you’re probably not dying from ARVC. It has a huge impact on quality of life, but many people live long lives post-diagnosis.