Complete Congenital Heart Block ?s

Hello...I am new here, and so glad to have found this website! I was just diagnosed two weeks ago with CCHB (complete congenital heart block) I am 38 years old. When I was 5, I was told I had a heart block, but no degree was assigned. Jump forward to ten years ago, and a regular cardio diagnosed me with 2nd degree wenckebach. He retired, I went into an EP, who appologetically confirmed that I had been MISDIAGNOSED all this time. He ran some tests, treadmill stress test (got up to 120 bpm max), echo (showed no enlargement and only mild valve issues), and holter monitor test (showed no pauses, 1000 extra beats over 24 hrs, rate averaged 48, lowest was 35 probably while sleeping). My EP was shocked at how "healthy" my heart was with CCHB. He suggested currently waiting to pace me, but said he'd put one in today if I was worried about the sudden cardiac death risk. I know my results are good, however, I still have CCHB...aren't I still at risk of syncope? I am looking for info on this condition and it is extremely limited. My only reservation is the fear of replacing leads...it quite frankly frightens me a bit. I thought all cases of CCHB required pacing. I have a tough decision to make...otherwise, my EP said he'd see me in 5 months, and then yearly for echos to see if my heart enlarges...

Advice? Please??? Thank you, in advance, for any help!

11 Comments

block

by Tracey_E - 2011-01-20 02:01:18

First of all, as far as I know CCHB is not related to sudden cardiac death. SCD is caused when the ventricles fibrillate instead of beating. Block is when the signal gets blocked between the SA node in the atria and the AV node in the ventricle. The primary reason to get a pm when you have a block is to feel better. If you are doing something active and your rate doesn't go up because of a block, you will be tired. If your rate drops off and is too low you can get tired and dizzy, and it's hard on our organs. If you do not have symptoms and you are able to get your rate up on the stress test, I would wait.

I have CCHB also and got my pm at 27 when my rate dropped from the 40's it had been all my life to the 30's, then the 20's. I felt fantastic after, had all my old energy back.

You can only predict syncopy if you have a crystal ball! If your rate stays where it is and you aren't dizzy, odds are pretty good you won't pass out. I never passed out even when my rate dropped to the 20's the day of surgery. I felt like crud, but I was conscious.

You can go in and out of third degree block. An occasional block is called 1st degree. 2nd degree is a complete block some of the time or a partial block all of the time. Third degree means complete block all of the time. It's possible to be 3rd degree but occasionally show up in 2nd degree or whatever so both of your diagnoses are likely correct.

Don't let leads be a reason not to get it! I'm 44 now and on my 4th pm. I still have one original lead, I had one replaced last time I got a new battery. it fit, so they just put it on top of the ones already there. If the vein gets too full, they either extract or go to the other side. It's something to think about, but not a primary consideration, in my opinion.

I wouldn't say all cases of CCHB need a pm, but it's pretty rare to get to your age and not need it. WhenI got mine at 27, that was considered pretty old for CCHB. Usually by the time we get out of our teens, our body stops compensating for the low hr and we begin to get symptoms. They usually catch it now on ultrasound so parents know before the baby is born if they have CCHB! And the babies often get pm's before they're a few days old. They treat it much more aggressively than they did when we were kids because pm technology is so much better there's no reason to wait.

syncope resulting in sudden death...

by sandk - 2011-01-20 02:01:35

Thank you so much for your reply...I appreciate your info...especially on the leads.

My EP is certain (verified by other EPs) that I am in complete congenital heart block 3rd degree all the time...no correlation between the upper and lower chambers.

As far as the sudden cardiac death from syncope - when I researched it, cardiosmart.org and mayo clinic had this to say...

"Third-Degree Heart Block - In this type of heart block, none of the electrical signals reach the ventricles. This type also is called complete heart block or complete AV block. When complete heart block occurs, special areas in the ventricles may create electrical signals to cause the ventricles to contract. This natural backup system is slow and isn't coordinated with the contraction of the atria. On an EKG, the normal pattern is disrupted. The P waves occur at a faster rate than the QRS waves. Complete heart block can be fatal. It can result in sudden cardiac arrest and death. This type of heart block needs emergency treatment. A temporary pacemaker may be used to keep the heart beating until you get a permanent pacemaker."

HOWEVER, I'd love to know where you heard that sudden cardiac death wasn't an issue for complete heart block, because this is the main reason I would GET a pacemaker...I am dizzy and fatigued, but I can deal with that for a while longer...



CCHB

by heartdoc - 2011-01-20 05:01:16

Cardiac standstill (ie, death) can occur in 3rd degree HB if the "backup system" fails. The fact that you are still around at 38yo, with no Hx of syncope, indicates that your backup is reliable. No guarantees, but I agree that it's not unreasonable to defer implant for a while. (You are apparently one of the lucky ones that has CCHB without any associated congenital structural abnormalities that can predispose to SCD.)
There are major advantages to having synchrony between the upper and lower chambers of the heart, and a pacemaker could restore this (assuming your upper chambers are still reasonably normal). You would probably feel better with one.
You should discuss the implications of right ventricular pacing (I'm assuming that's the kind of pacer he would use) with your EP. The significance of RV pacing depends on where in the conduction pathway your "backup system" is (something your EKG would show).

CCHB

by sandk - 2011-01-20 06:01:18

Thank you so much for your reply...it is sort of a tough decision, with the complications and surgeries that may/will follow. I think he mentioned dual chamber pacing...but I didn't hear him state RV pacing.

Thank you, again!!!

CCHB

by drashmore - 2011-01-20 08:01:09

I was recently diagnosed with Third-Degree Heart Block. What you copied in you above paragraph is exactly what they told me in the hospital? I walked in the with a HR of 36, went down while sleeping to the twenties. I never got dizzy or lightheaded but I was tired and started building fluid and having trouble breathing. This was all within a 2 day period. They put in a Boston S. with 2 leads, one to the right atria and ventrical.

same history

by sandk - 2011-01-21 01:01:24

That is so strange - but a relief, to find someone with almost identical history!

I have read that 90% of CCHBs test normal on the treadmill stress test...and from what else I have gathered, they truly have no single test that can predict if or when syncope or presyncope episodes can or will begin...of course other signs of damage such as enlarged heart, valves, etc., can show up. This is leading me to lean toward getting one. At least sometime this year. I think at 38, I just don't want to push my luck so to speak. I am having a REALLY difficult time finding others similar to me but older and without pacemakers. It is also a known fact that my backup vent. rate will drop as I age...

I did have over 1000 extra ventricular beats during the 24 hr holter monitor...does this mean I have a wide QRS rhythm?

I can't thank you all enough for sharing...CCHB seems to be so rare that medical journals and studies are far and few between - and outdated.

I think I have been thinking my fatigue is just normal...I am always the first one on the couch after dinner...maybe it is catching up with me a little.

Thanks again for all your input, I am very grateful.

for the one who sent a message

by sandk - 2011-01-21 02:01:03

Thank you, also, to the poster who sent me a message...I appreciate your story too! Through all my digging and researching on the internet, this club has been the most encouraging and useful of all!

drashmore

by Tracey_E - 2011-01-21 07:01:18

When you develop it as an adult, your body can react quickly and strongly. When you're born with it, like sandk and myself, your body compensates.

odd

by Tracey_E - 2011-01-21 08:01:18

I'm no dr so I could be missing something but if you are in 3rd degree block, there is no correlation between the atrial and ventricular beats which means your rate would not go up with exercise.

Follow up surgeries would normally be only when you need a new battery, every 5-10 years.

Complications happen less than 2% of the time, don't go by what you read here. For every one person who comes here for answers when they have a complication, there are hundreds or thousands out there who have never had a problem and therefore no reason to search us out.

If you are tired now, you would probably feel better with a pm. If you are able to do whatever you want now and be active and feel good, then I would wait.

My thoughts

by mgman - 2011-01-21 12:01:09

Hi Sandk,

I was in a very similar position to you. CCHB diagnosed at around age 5. Yearly check ups and Dr would always say to me as long as you feel ok then I'm happy but 1 day we might have to put a PM in if you get dizzy spells. I did exercise stress tests on treadmills and to the amazement of one of the nurses my rate did increase with exercise despite the 3rd degree block.

My annual check ups followed this pattern for years 'till I was around 24 when I saw a different Dr who raised the possibility of fatal stokes adams attacks (I think bascially blacking out and heart stopping). This was quite a shock to me as I'd always thought that as long as I felt ok I was ok.

We talked it through and he balanced the benefits of a PM against the very small risk of complications due to the surgery. He left the decision to me, but when I asked for his advice he said something along the lines of "If it were me and I felt ok, I'd probably push it another couple of years". And so I did.

I then moved to a new area, and upon seeing the specialist at my new hospital he basically said "Why haven't you got a pace maker? CCHB requires a PM". I explained that I'd never suffered any symptoms and that other Drs had been happy just to monitor the condition, and so we left it that I would be seen once a year.

This situation continued, but I think I secretly started to feel lethargic. I wouldn't admit it to myself, but I did start to read about the condition and about PMs - that is when I first found this site. I continued to push it to the back of my mind though.

Then last year aged 32 I had 2 episodes where I very nearly blacked out. I was at work (an office job, no exertion), and I turned on my chair to face someone when I felt I very nearly lost consciousness. Each time I felt a huge rush of adrenalin kicking in and I just stayed with it.

I put it to the back of my mind the first time, but when it happened again I went to my GP. He sent me directly to hospital where they admitted me immediately. The specialist said he wouldn't let me leave without a PM. My rate had been dropping into the 20s. It seems I should've listened to my body when I first started to feel slow and tired.

Since having the PM (now about 9months) I've never felt better. It was hard at first - the new faster rate felt like my heart was racing and I couldn't sleep. The tiredness made me feel sick, and for the first few days I felt rotten. Soon that passed and I began to feel normal. Now I have so much more energy and peace of mind that I have less chance of stopping beating. The scar is very small and neat and you really don't see a lump where the PM is at all.

I worried about how other people would perceive me with the PM. Would they think me weaker even subconsciously? Would they treat me differently? That hasn't turned out to be the case at all. Sure the first few days back at work people all had a lot of questions and I told the same story over and over, but now I'm just me again. I honestly think people have mostly forgotten, they just see me as a regular guy like I always was.

My advice is not to worry about any negative aspects of having a PM, for me they're so insignificant they're just not worth thinking about. It feels a part of me now - I don't even think about it most days, but when I do knowing I have an amazing bit of technology inside me helping my body function better is a pretty cool thought.

Good luck with whatever you decide,
Phil.

similar story

by jwhalen - 2011-04-02 03:04:43

Hi MGMAN
I have had my PM now for 3 weeks..complete heart block only picked up by my event monitor on the last week of wearing it.
Loved reading your story and it made me feel less anxious about the whole thing. i am returning to work soon and wonder what all my co workers reaction will be.
Thanks for your support.
JJ

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But I think it will make me feel a lot better. My stamina to walk is already better, even right after surgery. They had me walk all around the floor before they would release me. I did so without being exhausted and winded the way I had been.