Myeloproliferative Neoplasms/Polycythemia


I am wondering if any members have a myeloproliferative neoplasm (MPN) such as polycythemia vera (PV). If so, have your doctors made any connections between hypercoagulability and your heart condition? Did your MPN status change anything about your diagnosis, pacemaker installation or programming?

I have polycythemia vera and was diagnosed with sick sinus syndrome in July 2019. I had a single lead atrial PM installed then. My resting heart rate has been very low for years (always under 50, more typically 40s) then it got lower and lower until it stopped. 

The cardiac folks and the hemotology folks don't seem to talk to each other and neither group seems curious about a possible interaction of these diseases or different approaches to treatment. In questioning my cardiac surgeon just before implantation it was decided to switch me from a dual-lead to single-lead to reduce potential clotting issues (which PV folks are prone to). But this decision was based on very limited information and I am still not sure if it was the right thing to do. 

I would be grateful to hear about the experiences of other MPN patients. 



Interesting thought.

by crustyg - 2019-11-11 13:21:51

There's a clear link between myelodysplastic syndrome and cardiomyopathy (it's another cause of iron poisoning of the heart muscle) which is why *everyone* presenting with AFlutter or SSS gets a ferritin level done.

Whether PRV (to use the older name) is causally linked to SSS I'm not sure - but some cases of PRV can change over time to become a myelodysplastic syndrome.  It's not great.

And to be fair to us useless communicators, AngySparrow, speculating about this sort of linkage isn't usually helpful as Sinjawns may agree about.

It's certainly not great installing leads into patients who are hypercoagulable.

Research links?

by sinjawns - 2019-11-11 14:33:37

Thanks for the welcome and the comments.

I don't mean to suggest that my physicians (or all physicians) are poor communicators. The issue is more about the disciplinary silos and a lack of interest (time?) to work collaboratively in complex cases. 

I'm looking for both patient experience in this area and research evidence. CrustyG, can you point me towards any recent research on MPNs and cardiomyoptathy or SSS? The only relevant study I have found was this one:

Thanks again. 


by Gemita - 2019-11-11 16:55:23

Hello Sinjawns, you are a complex case and I wish I could help you.  

Yes I have found that with complex health issues, specialists tend to stick to their own area of expertise and leave overlapping health issues to the appropriate speciality.  I recently had a problem with my gastro team who wouldnt fully liaise with my cardiac team.  I felt certain my heart arrhythmias were oesophageal related but all my gastro team could say was get the arrhythmias fixed by an ablation and we will deal with your oesophagus.  My feeling is that after an ablation my oesophageal motility issues will still exist and therefore be a strong trigger for my arrhythmias. 

As far as a single lead pacemaker is concerned, I was told it was more "physiologically natural"  to have a dual lead pacemaker and also that a single lead to right ventricle alone may lead to left ventricular systolic dysfunction, requiring an additional lead to assist left ventricle.  I note however Crustyg's comments in regard to your condition and about the potential dangers of installing leads, so I guess they have taken the easier path to try to help you.

I do wish you well and that you will find some answers.  

For those with good AV conduction, atrial pacing is better than ventricular

by crustyg - 2019-11-11 17:32:38

There's a clear geographical fashion for pacing: for a patient with SSS in Scandinavia with good AV conduction no EP doc woud insert two leads.  In USA, they always would, if I understand correctly.  But now the evidence is growing that RV apical pacing is bad for some patients so His-bundle pacing has been re-activated (pun intended), as it avoids the LV remodelling associated with RV apical pacing and takes care of the time when AV conduction becomes an issue (it doesn't always).

It will be some time before I can hit the literature Sinjawns, and even then I suspect that it's going to be thin - it's going to have to be national registry based, I think.  The study that you quoted relates to stroke in Afib and I think has been superseded by CHADSVASC and now CHA2DS2 scoring for formal anticoagulation.

There's a very good reason why practising doctors keep their own counsel about areas outside their own area of specialisation - the risk of saying or doing something that *was* correct back in the day, but which is now known to be incorrect or has been superceded.  I keep asking myself if I'm making that mistake...  Just as well that I'm not practising.

However, it does seem pretty clear that some of the forum contributors have had personal experience of the super-specialist who doesn't look at the bigger picture.  You don't have to be a True Believer in holistic medicine to see that a more joined up approach might sometimes be better.  I suspect that as we increase our understanding of the true scale of the genetic abonormality burden that we humans carry, we'll have to spend a little more time thinking about true root causes (i.e. it's a gene defect underlying the disease) or manifestations of the same underlying disease process(es) in other organ systems which aren't our primary area of expertise.

Lower oesophagus/gastric problems causing *some* heart rhythm issues

by crustyg - 2019-11-11 17:47:31

Gemita: I don't see any real problem with believing that runs of PVCs might be caused by lower oesophageal or gastric issues.  The anatomy is pretty close and some of the innervation is the same.  What anyone might actually *do* about it is perhaps more of a problem.  I don't think we'll find any studies linking oral acid-neutralisers to reversal of AFib, for example (daft example chosen for effect).  But excessive sodium bicarbonate *can* tip a patient into heart failure, so it's not totally mad as a wacky suggestion

So you may be right. And separating triggers from an underlying tendency towards arrhythmias is not going to be easy.

Thank you

by Gemita - 2019-11-11 19:51:00

Dear Crustyg, yes I am very much into finding the root cause of a problem and treating that, not just the symptoms. But symptom relief is certainly easier and cheaper to achieve.

I don't want to hijack this thread but thank you for your helpful thoughts on my personal situation. I have a connective tissue disorder (Ehlers Danlos) and I have always suffered from severe gastric motility issues from start to finish !!  The spasms in the oesophagus and elsewhere can really trigger AF and other arrhythmias although I accept I now have the substrate for AF so even if I fix my gastric issues the substrate in the heart will remain and I will be prone to further episodes.



Why Should...

by donr - 2019-11-12 00:31:03

...any "superspecialist" ask questions that lapse over into another speciaty?  Bec ause more than rarely something happening there may affect his/ger work.   I see a Cardioo, DI, Allergy/asthma specialist.  Let me give you a personal couple examples. 

1) I have GERD.   That is the primary area of expertise of my GI.  But my Allergist, who treats me for asthma tells me that GERD can very severely cause/potentiate asthma. So, I tell my GI exactly what my allergist is doing & how he has been concerned about it before my GI & I met. 

2)  I had a collectomy in 2014.  I have random bouts of what I call explosive diarrhea.  I have also developed some pretty messy random bouts of errhtyhmias.   I had a PM download last week that disclosed  that my arrhythmias were not just randomly distributed - they came in bunches, with periods of no ne whatsoever.  I mentiioned this in another comment on another thread.  I was talaking w/ my retired Cardio nurse friend who used to work for my long term Cardioi (16 yrs), who is also a reasonably decent EP.  She noticed the strange distribution of my arrhythmias & asked me if the Cardio asked me about diarrhea.  I sain NO, & ididn't volunteer it.  She suggested that the diarrhea could cause hypokalemia, which can cause arrhythmias.    You have chronic hypokalemia.  He needs to know.  She suggested that I have a glassful of Smoothie once a day, made w/ high K fruit.  Can't hurt, & it might clear up the prroblem.  Well, after a week, I at least haven't had the Galloping Trots, so that problem is gone - will wait to see about the hypokalemia.  Apparently diarrhea can mess up electrolytes quite rapidly & even though I toook 20 mil EQ of potassium, it may NOT have covered my diarrhea lost Potassium.

But why should any one of those three start asking about overlapping problems?  When you are constrained to 15-20 min per patient by the admin poers that be, you just don't get time to think of off the wall issues. 

3)   Our Daugghter is a PCP.  She gets into these things that overlap.  She has to, because she gets to see patients who stump the specialists.  I have ard her wander far afield into many specialties when talking w/ patients.  When I questioned her later she starts wandering into all thoise specialties and comes out with good reasons. 

4)  I have had 3 exposures to Rabies - all required the shot series.  I was at a loss as to what to do after the first (Exposure to coyote brains) so I went to talk w/ our vet.  She loaned me her CDC Rabies treatment manual & fortified by a bit of knowledge, went to three ER's.  I got some of the stupidest answers known to man.  The third ER Doc told me to drop my drawers & lie on my belly - I was about to get my first shot in the butt.   The second had said he didn't think I had any wories from my description of the incident.  The first I cannot recall.  Doc 3 gave me the correct answer according to CDC protocol.  The CDC manual criteria for Bats is that ANY physical contact w/ a bat , live or dead, is considered a rabies exposure.  I have had two exposures when a bat flew through my hair.   Fortunately both times the first ER Doc I saw knew the CDC answer.    My point - fopr Rabies, a multi-disciplined doctor is necessary to get proper treatment.  AND - they have to be practicing in an area where it exists or they will mot know about it.  My vet put it quite simply - you have to be your own gatekeeper.


Great post Donr

by Gemita - 2019-11-12 03:57:14

I enjoyed reading your post. Yes it is largely a problem of time constraints, shortage of doctors and not enough financial support in our health system here in the UK although fortunately there are still doctors who will go that extra mile to get to the root of the problem.  This is why it is so important to be our own advocate and to keep searching for answers because no one knows our body as well as we do.

Donr, the potassium problem - can't a good doctor see evidence of this on an ECG so a "watchful" clinic with good resources should have picked this up. No wonder so many patients take matters into their own hands and buy monitoring equipment to pass recordings on to their consultant. 

Potassium Problem

by donr - 2019-11-12 10:48:45

Genita:  Potassiun (K) is something that can only be found by taking a blood sample & running it through the lab.  That's true of all the electrolytes - like Magnesiun,Sodium, for example.  The ECG will give you a hint that it is a problem, but you have to then go the blood sample route.  At least that is the case today.  To really find out if it is a K problem, youneed to have a series of blood tests to match up w/ a PM download report to find out if the low K & arrhythmias line up in occurrence.  Both tests cost money, take time & require that the patient go to the places where the data can be taken.  So, no one is real anxious to do it.  In my case, I just happened to have a K test when I was having a bunch of arrhythmias.  My K level was very low - 3.2, if I remember correctly.  It should be in the mid 4 range.   So they came in with a pill the size that would ckoke a horse.  Diud it help?  Neither the hospital nor I know because they did not run another PM download to find out.  Matching cause & effect is not always eassy - especially when there may be multiple causes for a given effect.


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Member Quotes

I had a pacemaker when I was 11. I never once thought I wasn't a 'normal kid' nor was I ever treated differently because of it. I could do everything all my friends were doing; I just happened to have a battery attached to my heart to help it work.