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Heart Disease  (Expert Forum)
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Neurocardiogenic Syncope w/AV Block vs. Orthostatic Intolerance
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Neurocardiogenic Syncope w/AV Block vs. Orthostatic Intolerance

by SingingFish, Mar 27, 2005 12:00AM
Female, 41, 5’4” 120, daily exercise. Years of fatigue & discomfort standing/sitting still. Seated BP 90/52, HR 54 bpm; 42-44 bpm asleep. Lying-standing, HR rises 20-60 bpm, BP falls104/60->75/53. Lowest I’ve seen: 54/45. No meds. Family heart history.

Some syncope & near syncope, which I often abort. Daily presyncope. Sitting 15-20’ causes symptoms: eg BP 80/50 w/HR <50, my brain won’t process. I’m a classical singer: long phrases induce dizziness, nausea, pallor, chills, sweats. Vision blurs. Some chest pain, brown outs.

24-hr holters: sinus tach w/symptoms (while singing: 131 & 142 bpm, BP undetectable). Rare ventricular ectopy, v rare supraventricular ectopy.

EKG’s: borderline, L atrial enlargement, bradycardia. Stress echo normal.

Vanderbilt neurologist noted elevated standing serum norepi, hypotension, OI, pectus excavatum. Suggested salt, hose, biofeedback.

3 biofeedback tests: 1-3X/min standing HR rose >150-200 bpm, fell to 32-37, stabilized @ 75. Suspecting arrhythmia, doc referred me to her EP.

EP assumed NCS. Tilt test: I passed out @ 13’. While out, no pulse or BP detected & EKG showed 2:1 AV block, primarily cardioinhibitory, 2.5” btw QRS waves. EP suggests pacemaker, multiple opinions. 2nd cardiologist questions tachy-brady.

How do I distinguish NCS vs. OI? How does singing cause tachycardia & near syncope? What’s detected by biofeedback equipment & not recorded by other tests? Can syncope trigger AV block? When is a pacemaker warranted? Would it alleviate fatigue, discomfort & brain dysfunction sitting still? How should I choose docs for 2nd opinions?

by CCF-M.D.-MJM, Mar 27, 2005 12:00AM
That is quite a history.  It certainly sounds like neurocardiogenic syncope.  One key is to look at your initial response as you are developing symptoms.  If the first response is tachycardia before hypotension, a beta blocker can help.  The reason beta blocker can help is the sensor in your neck and brain detect an acute change heart rate and incresase your vagal tone, which acutely slows down your heart rate and may lead to the drop in blood pressure. It is often to difficult to tell which one occurs first.  I trial of beta blocker may help tease this out -- it will either help or it won't.  If it doesn't, then stop it.

I don't think the singing has anything to do with it unless you are developing stage freight for the first time in your life or panic anxiety -- this would be a pretty extreme case of that though.

Biofeedback is way for you to watch your bodies response tachycardia and learn to try to control the symptoms with relaxation techniques or positive thinking.  It dramatically helps some, but not all.

The AV block you see is either from intrinsic problems with from increased vagal tone related to the tachycardia or you it could be sick sinus syndrome.  From what you write, it sounds like increased vagal tone related to NCS, but an EP study would help clarify if there is any conduction disease in your heart.

Indications for pacing per ACC/AHA/NASPE guidelines published in (Circulation 1998;97:1325-1335.)  include

Indications for Permanent Pacing in Hypersensitive Carotid Sinus Syndrome and Neurally Mediated Syncope


Class I


1. Recurrent syncope caused by carotid sinus stimulation; minimal carotid sinus pressure induces ventricular asystole of >3 seconds' duration in the absence of any medication that depresses the sinus node or AV conduction. (Level of evidence: C)

Class IIa


1. Recurrent syncope without clear, provocative events and with a hypersensitive cardioinhibitory response. (Level of evidence: C)
2. Syncope of unexplained origin when major abnormalities of sinus node function or AV conduction are discovered or provoked in electrophysiological studies. (Level of evidence: C)

Class IIb


1. Neurally mediated syncope with significant bradycardia reproduced by a head-up tilt with or without isoproterenol or other provocative maneuvers. (Level of evidence: B)

Class III  (THIS MEANS PACING IS CONTRAINDICATED)


1. A hyperactive cardioinhibitory response to carotid sinus stimulation in the absence of symptoms.
2. A hyperactive cardioinhibitory response to carotid sinus stimulation in the presence of vague symptoms such as dizziness, light-headedness, or both.
3. Recurrent syncope, light-headedness, or dizziness in the absence of a hyperactive cardioinhibitory response.
4. Situational vasovagal syncope in which avoidance behavior is effective.

Pacing can help prevent people from syncope, but more importantly it can buy an extra few seconds before syncope to allow you time to sit down and avoid injury.

Would it alleviate fatigue, discomfort & brain dysfunction sitting still?

this varies from person to person, there is no way to know for sure.

Regarding a second opinion, most academic centers will have someone that specializes in this arena.  When you go for a second opinion, make sure you take all your old records to save time, including actually ekg print outs and not reports of the ekg, actual echo, as much of the tilt table data as they will give you.

Regarding orthostatic intolerance (OI), this is not a term that I've seen used very often, but from what you have written, I do not think it applies to you.

i hope this helps.

good luck.

Member Comments (9)

by SingingFish, Mar 27, 2005 12:00AM
To: CCF-M.D.-MJM CCF-M.D.-MJM CCF M.D. MJM
Dear Dr. MJM,

Thanks so much for the incredibly speedy and detailed response!  This forum is terrific!

“One key is to look at your initial response as you are developing symptoms.  If the first response is tachycardia before hypotension, a beta blocker can help.”

I think the BP drop precedes the tachycardia. I occasionally check first thing in the morning, particularly if I’ve been in bed for 7+ hours, when symptoms tend to be worse. The patterns are pretty consistent. Today, for instance, with an arm cuff, my BP lying down was 104/63, pulse 47. Immediately upon standing: BP 79/61 HR 69; After 2’: BP 96/64 HR 78; 2’ later 83/61, HR “error.” 2’ later all readings “error.” Having stood for 6 minutes, I was uncomfortable & lay back down. BP sometimes measures 66/48 and/or HR 100+, but I usually walk before that happens.

Both the neurologist at the Vanderbilt Autonomic Dysfunction Center & my Illinois EP initially considered beta blockers. After the tilt test my EP changed his mind, saying that given my AV block, 2.5-second asystole & daily low pulse of 42 bpm, if I were to “break through” while on a beta blocker, it could be "life-threatening."

“I don't think the singing has anything to do with it unless you are developing stage freight for the first time in your life or panic anxiety -- this would be a pretty extreme case of that though.”

I doubt it’s stage fright. Singing consecutive, slow phrases, especially in a high tessitura  (requires more rapid exhalation) triggers syncopal symptoms consistently -- whether I’m on stage at Carnegie Hall, singing hymns in a church service or practicing at home. I can alleviate symptoms somewhat by walking while singing or omitting occasional notes. In songs with rests between phrases, I’ve recovery time, but when only quick “catch breaths” can be taken, I’m in trouble sooner. Does anyone have similar experiences with singing or giving speeches, or know about “trumpet player syncope?” The Vanderbilt neurologist cited increased intrathoracic (?) pressure & my EP cited decreased venous return to the heart, but my understanding of these concepts is limited.

“Biofeedback is way for you to watch your bodies response tachycardia and learn to try to control the symptoms with relaxation techniques or positive thinking.  It dramatically helps some, but not all.”

After the initial heart-rate testing, the biofeedback doc adamantly refused to teach me biofeedback techniques. She saw that I was relaxed & told me the techniques could be dangerous, given the pronounced arrhythmias her equipment detected. Other doctors are puzzled by the consistent & dramatic shifts that show up on her equipment.

“Pacing can help prevent people from syncope, but more importantly it can buy an extra few seconds before syncope to allow you time to sit down and avoid injury.”

I have minutes’ warning before syncope, so I can abort it (except on a tilt table!). Would a pacemaker still help me? I wonder whether my AV block is potentially dangerous. Also what might improve the daily discomfort and cognitive problems I have when standing or sitting still. Sitting in an audience has become extremely uncomfortable!

Thanks for recommending I request full EKG, echo & tilt print outs, not just summaries! I’ve tried. How do I persuade Medical Records & my EP, who told me the 7 excerpted strips from the tilt should be enough for 2nd opinions?

Happy Easter to all!
SingingFish

by Emmaj, Mar 29, 2005 12:00AM
To: Singing Fish
Hi,

I feel totally inadequate responding to you when I know nothing about these matters, but... Your symptoms sound truly nightmarish and you are young too. A pacemaker, if it weren't contraindicated, could keep your hr up, say over 60 bpm, and it seems to me that alone would make you feel better and perhaps allow you to take a beta blocker... (although I am clueless what that would do to your bp). Sometimes on this forum, the doc suggests that a patient wanting a second opinion visit so and so at CCF. If you could afford it, the best: CCF or Mayo? On the clinic websites, you can find dr bios that indicate the drs' main medical interests. Best of luck!

by salis110, Mar 30, 2005 12:00AM
dear everybody
somehow I have same symptoms, sometimes i feel dizzy but when i check my pulse it is normal as always for example it is 60 or little more or less.but sometimes it beats faster till 90-100 without any activity, at this period of beats i feel something happens in my chest, it is not pain or ? and after a short time (1 or 2 secs)i feel a heavy pulse and after that every thing goes ok but some anxiety.If casually through this event I check my pulse I feel an interrupt or skipping pulse.It makes me crazy.Also sometimes i feel pain in my chest bones dr.s siad they are due to ur anxiety.
I did echo which was ok and 24 hrs holter i had a period of 43 bpm during sleep at night and a 2.14 secs rest in hearts beat plus 2 rests less than 2 secs.
whats going on me? what are skips in my heart with odd feeling?
I am 27 yr/male ,173 cm and 73 kgs casually sport(soccer)

by SingingFish, Mar 31, 2005 12:00AM
Hi Everyone,

Salis110, I can understand the frustration of knowing something is amiss yet hearing from physicians that your tests are simply "borderline." After a year of persistence and many tests, I finally had a tilt table test that "captured" a significant abnormality. By recording the data myself and noting the situations that consistently trigger changes in BP and heart rate, I've gotten better responsiveness from physicians. I've also found it helpful to approach physicians with a clear list of specific questions. Hang in there!

emmaj, thanks for the encouragement to pursue Cleveland or Mayo, I'd been thinking along those lines, looking for a tertiary care center that has a team approach. (Especially because I have perverse glucose tolerance test results -- sugar declined initially. Also odd results on sleep studies -- 9% REM and sleep architecture anomalies. My hunch is that these issues all relate in some way.) The assessments & advice I've gotten from individual specialists is so divergent that it seems time for a coordinated approach.

Question for those of you who have had success with going to Cleveland, Mayo, or Johns Hopkins: How have you chosen your physician(s)?

fwilson: thanks for the performance suggestions! Yes, I've used those ideas while standing & singing -- all sorts of muscle exercises are helpful: bending knees to use quads, curling toes, clenching fists, etc. also quietly hyperventilating during rests. You mentioned "stretch," a term I've seen before but don't understand. What is this? Have you any suggestions for learning more about baroreceptors and chest pressure?  What is your instrument?

Best wishes to all,
SingingFish

by Emmaj, Mar 31, 2005 12:00AM
To: Singing Fish
Hi SF,

I actually went to Mayo (live in upstate NY) on the recommendation of my cardio here... He wanted me to see Rick Nishimura. My experience at Mayo with Dr. Nishimura was very positive -- RN said he had spent longer researching my particular issues than he had for any of his patients in a long time... which made me feel really good (only because my situation is not straightforward). He is a brilliant man, and at the same time, very approachable and "human." He is not an EP, however. A woman I work with went to CCF, and her experience there was very positive, but I'm not sure of names offhand... she probably does have an EP there, so if I find out the name, I will try to post it for you. You might have better luck finding info on particular docs at the valvereplacement.com forum... I say that only because many people frequent that forum and probably a fair number have arrhythmias and will jump to answer your questions about docs... given the way that forum is organized. Best of luck and hope you find lots of encouragement and answers!

by Emmaj, Apr 04, 2005 12:00AM
To: Singing Fish
Hi again SF,

I did find out the name of the EP my coworker sees at CCF: Wael Jaber. She says he is wonderful. I do not know of any other names at Mayo or Cleveland (except my wonderful surgeon's and I know you are not in the market:), but I hardly think you could go wrong with anyone at either place. Good luck!

by Emmaj, Apr 13, 2005 12:00AM
to set the record straight... i learned that wael jabber is a great cardio, though not ep by subspecialty...

by criehaven, Jun 12, 2005 12:00AM
Hi All:  Thrilled to have found this site.  I was dx with sick sinus syndrome this week... pulse between 30 and 40, very low blood pressure, crystal clear arteries (that heart cath was no fun, and truly set off the symptoms of neurocardiogenic incident.)  I am having an appt to meet with the pacemaker surgeon in two days, and feel like I need to make certain he understands that NCS issue is deeply contributing to my chest pain, fatigue and three to five times a year passing out.  I am amazed to have found this info, as I have suffered with this random passing out since I was a teen (I'm 41 now).  Concerts, baseball games, parades, any crowded situation (though I never felt panicky about crowds or was agorophobic).  Pain is another big trigger.  I know I set off the hospital's bells and whistles while hospitalized last week.  I got up and tweaked my incision, and it  hurt, and the nurses all raced in to see if I was dropping out of consciousness.  I do have a past history of adrenal exhaustion, and recall that the tilt test was in some way significant there as well?  Mostly, I am very very relieved that I have reason to not reduce my salt intake... I KNOW in my soul I need it, and always have, and truly the fear I was having around my heart issue was that I  might have to cut back.  Whew.  

So, questions I have are: what should I be asking my pacemaker doc about?  Do I need to see anyone and get further diagnosis for NCS or can I get away with the non-medical measures?  (I've gotten pretty good at feeling the drop coming on, and am not shy about lying on the ground with my feet up.)  Should I be concerned with my exertional output?  I am a horse trainer, and was a toplevel ski racer as a teen, so have always had a very active, long hours, steady physical output (sometimes intense, as I work with young horses almost exclusively) for 20 years.  The past eight years have seen me unconsciously cutting back and changing my show schedules and routines to accommodate increasing fatigue.  Now am virtually unable to do my job at all.  Wondering if the pacemaker will address the NCS as well.  

Thanks for your thoughts and ideas.  I am scheduled to see Dr Chen, who is the late effects cardiologist at Dana Farber (I had non hodgkins lymphoma at age 19, and need to be evaluated for chemo effects anyway) but it sounded like the Beth Israel Hospital has a neurological center, adn they are right next door... anyone know of it, or have any docs to recommend from there?  

Katrina B Crie
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