CPVT? really?

[cookiemom4]

I am a healthy(?) 35 year old woman hoping for a little guidance on my recent diagnosis.  Ready for the life story?
For a few years now I have wondered how normal it is for me (who is in good but not phenomenal shape) to have such a slow heart rate (39-43) when I didn't seem to have that in my more fit younger years.  While at the family Dr. for a sinus infection I happened to mention it to him and he suggested the I see a cardiologist just to check it out.  I didn't.  At least not until I was training for a half marathon and my heart started to skip beats occasionally.  With that extra excitement I decided it would be a good idea to have it checked out.  The cardiologist didn't seem to be concerned but ordered a stress echo to rule anything out.  During the test I only had one skipped beat (about 15 minutes into the test) and the result of the test was a boost to my ego as they told me what great shape my heart was in and that I would probably live forever.  Great!  That is until a few week later while running my heart went CRAZY.  My heart rate monitor (that I wear for training purposes) was picking it up around 200 bpm (my average running HR is 143).  It probably lasted for about a  minute and then I just started coughing and couldn't stop.  It REALLY scared me- and the girls I was running with.  As soon as I got home I called the cardiologist and he ordered an event monitor.  During the 2 weeks I had it I had a bunch of "skipped" heart beats but nothing else.  The Dr. reassured me that I was safe to run in the race even though I was very nervous about it.  Because I was feeling so unsure he decided that I should see the electrophysiology cardiologist.  So I did!  This last Wednesday I sat down with this new Dr. expecting him to tell me that I was having atrial arrhythmia and to chill out- but that is definitely not what he said.
He explained that he wasn't too concerned about the low HR but that the arrhythimias I am having are PVC's and the event monitor picked up one couplet.  His concern was that they come on when I am at the peak of the workout rather that when warming up or cooling down.  His diagnosis:  CPVT minus the VT but with PVC's.  I have no family history and no history of syncope.  He put me on Atenolol 50mg but after I expressed concern about having even lower BP (normal is 90/60) and lower HR he told me I could start with half a tab and see how it went.  He told me that if they didn't stop to just up the dose. He also ordered another event monitor and told me to continue exercising.  After doing my research, that seems a little counter intuitive!  I certainly don't want to give up exercising but I am not really in the mood to drop dead even more!
So, it has been 4 day on the beta blocker and I am riding to church today when my heart decided to do some flip flopping around and I felt really weird.  I am sure that it went 3 or 4 rounds before I had to cough.  No stress, no exercise.  Of course the event monitor won't be here until tomorrow! What the heck is going on with me?  Any ideas?
Thanks!  

[Aleksandra72]

Hi,
I agree with the last comment. I have CPVT (not known gene though) and I have an ICD. To place an ICD, it is not a complicated procedure: local anesthetic and it lasts couple of hours. I also had to get my "cable" changed twice (unfortunately) and after 8 years the battery run out so the generator was changed 2 years ago. Otherwise I have to say that it is easy to live with ICD in daily life = without having constant fear of sudden death...

I have had betablockers since I was 18 (now 40). Their worst sideaffect is the some level of constant tiredness, unfortunately. Otherwise they are still quite harmless medicine compared to other options.

[is_something_wrong]

Hi,

As you probably know, CPVT is an extremely rare condition. It affects approx. 1:10.000.

Treatment is so-called nonselective beta blockers (Nadolol or Propranolol). Nadolol has a longer effect, with Propranolol you need to take the medication several times a day or take extended release capsules.

Some CPVT sufferers need to implant an ICD, especially if runs of ventricular tachycardia (bidirectional ventricular tachycardia, the hallmark sign of CPVT) is documented. Implanting an ICD is not heart surgery, it's done when you are fully awake and it takes approx. an hour.

An unfortunate effect of beta blockers is the mentioned sluggishness and laziness. However, it's critical that your heart rate does not go above a certain level, or else you'll get lots of PVCs which may degrade into a ventricular tachycardia. It will happen with adrenaline excess.

As you probably know, you should avoid certain activities like swimming. And make sure to follow up with your doctor on regular basis, and if you ever experience severe palpitations or fainting, see a doctor immediately.

[SamRobinson]

Hi, I have CPVT too.
I am 19 now but was diagnosed when I was 13 after finding out my mom had it which caused her to go into cardiac arrest at least 10 times before she was 35 she now has a defibrillator. Me, my sister and brother were tested and me and my sister got diagnosed.
I haven't had any heart problems until now I seriously thought I never had a heart condition and in the past year I have palpitations almost once every 2 weeks it scares the hell out of me after seeing my mom pass out and I do not want to have a defibrillator and go through heart surgery. My mom had to go through it twice within 5 years because of Medtronics faulty leads they put in her!
I take 40 mg of Nadolol (a beta blocker) every day and it seemed to be wroking awesome until now.
I was just curious to what medication you guys take? Mine makes me very slugish and lazy.

[Ratha110]

HI there,

i have CPVT, i have had since birth, and i have had 6 heart attacks. And 1 1\2 i had my 1 st operation, and now in my left shoulder i have an ICD. i understand what you are going thought. i hope you that you can get back to excursus!

[cookiemom4]

Thanks for taking the time to comment!  It is good to hear that someone else is managing with the stress of this crazy diagnosis.  I talked to the EP on Wednesday and he backed up a little and told me that he can't be sure of the CPVT with such little information.  I don't know if I feel a whole lot better since it doesn't seem like the diagnosis is going so well.
I am wearing an event monitor and taking the beta blockers but it seems like the beta blocker are doing such a good job that now I am not having any PVC's because I can't get my HR above 140.  Seems a little counter productive to be working out so, so hard to get some symptoms going but at the same time preventing the symptoms- to prevent sudden death.  Hmmm.  The only symptom I am having is my HR being so low that Lifewatch keeps calling me to see if I am okay- usually in the middle of the night! grrrrrr.
I have a couple of thoughts.  Would it be best if I go off the meds to get something diagnosic, or stay on the meds to prevent anything and just assume that I am a ticking time bomb?  I am a little concerned that with my HR being so low I am going to exercise my guts out and gain weight because I can't burn anything off and then get some obesity related heart disease. (okay, a little neurotic- but possible, right?)

How did they know that your husband had the disease?  What led them to do the EP study?  I am in Virginia so I am not too far from Johns Hopkins but the info they are working from is so minimal that I don't want to waste the time if there isn't some foundation.  

[cpvtwife]

My husband (35) has CPVT, recently diagnosed.  He also has a low resting heart rate but has very high heart rate with adrenaline (220+).  The doc should be able to do an EPS (Electrophysiological Study) and inject adrenaline and watch heart rhythms to confirm the CPVT diagnosis.  Depending on where you live, there are some docs I can recommend.  We just got back from visiting Mayo Clinic in MN where one of the US experts (Dr Mike Ackerman) works.

My husband is on high doses of beta blockers but also had an ICD implanted because his heart rates still go high despite beta blocker.  I think there are varying severities of the disease.

[Mom2four85]

That happened with me on Toprol, even a 1/2 of a small dosage.  I had to go to the ER and get a shot of something [brain fog] which stabilized my bp 70/40 and hr 41 for 11 hours and then they sent me home because they said I was stable :P  advised me not to take Toprol again.

I have a really sensitve system and can't take meds without some side effect - after surgery I was put on Bisoprolol which was horrible and I finally went off them and got my EP to give me Inderal to take as needed instead of daily.

Hopefully they'll find what's going on with you.

[cookiemom4]

Thanks for taking the time to comment.  I have had a few more PVC's but nothing else exciting.  If I had normal heart rate and BP I would just follow the Dr's advice and up my beta blocker.  However my HR is at 38 and my BP is 80/48 so I don't dare take any more.  Off to see the EC tomorrow.  Hope he has happy things to say!  Maybe the fact that the PVC's are happening at rest now will rule out CPVT. ?  Hmmmm.

[Mom2four85]

I've had both non and sustained VT; plus a multitude of other arrhythmia's - sometimes it's difficult to know what I'm feeling when - the only way I know is when it shows on my test results and I say AHHH that's what that was.

I do know from my stress treadmill test I had both VT and syncopal events that made me take a very inoppurtune nap :p  But then again, I faint at the drop of a hat with no provocation due to ANS (autonomic nervous system) problems I have, so I have a double whammy.

I think the cough was your body's way of putting your heart back on track - I know mine does sometimes and I had read about that manuever and did it one night when my heart wouldn't stop racing and it worked.

Arrhythmia's are so vastly different in each of us, it's hard to say if that's what you experienced.  

Certainly get your test results and ask questions and even think of another opinion from another EP would be in order.

[itdood]

VT is technically > 3 PVCs in a row.

If the VT terminates in < 30 seconds it's called none-sustained VT.  If it last longer than 30 seconds it's sustained VT.

People can be in VT and not get syncope.

[cookiemom4]

Thanks for your comment!  CPVT is apparently an ion channelopathy and is also known as sudden arrythmia death syndrome.  He explained it as the syndrome many athletes have that drop dead for no apparent reason while playing sports, etc.  Typically it affects children and adolescents.  
Knowing that you experience VT's, do you think that is what I experienced while running that lasted for about a minute and made me cough?  Not sure if that is what he is thinking.

[Mom2four85]

Sorry to hear you're going through this, I don't have any knowledge of CPVT; that's a new one to me - what is it?  

Thankfully you're not having the VT part of it; especially during exercise or exertion - it makes me pass out when I do.  That's one of the hardest things for me; going from a normal "healthy" routine walking/running and a full schedule to barely being able to function.

Did they do a stress test with you hooked up so they could get the results during exertion?

I would get all my results from the dr's you've seen and see what they say.  It sounds like more is going on but I'm only a patient and can only relate what's been my experience.  Then; after you get your results you can always get another opinion even if it is just to ease your mind if nothing is wrong.