Long QT syndrome

[valger59]

Hi~my daughter is 13 years old, and today I took her in for a physical to do cross country. The doctor said he heard a faint murmur, and so gave her an EKG.  Then, he came in saying the machine said she had "borderline Long QT syndrome".  He went on to reassure me that the machines have been doing that alot, and maybe they are more sensitive than they should be, that they have strict standards, and it still has to be interpreted by a doctor.  We have no family history that I know of, I get heart palpitations, but don't have long QT according to my cardiologists, just benign PVC's.  Is it common for these machines to interpret things wrong?  I am so worried, she is fine otherwise, other than the fact that she wears a Scoliosis brace right now, for moderate scoliosis.  She does balllet four days a week, and is generally active, and healthy.  Thank you so much, Val

[Michael J. McWilliams, M.D.]

Is it common for these machines to interpret things wrong?

Very common.  I only look at it after I have finished reading the EKG.  The QT interval is hard to read  sometimes and it does take some experience.  It is helpful that there is not family history of sudden death, seizures, drownings or sudden infant death syndrome.  If there is any questions, it is always worth seeing a cardiologist.  Sometimes a simple exercise stress test will help answer the question.

Murmurs are not related to long QT syndrome.  Even if she had a murmur, not all murmurs are bad.

I hope this helps.  Thanks for posting.

[valger59]

I'm sorry, one more thing I forgot to mention, when she was an infant, we had to take to to an ER for something unrelated, and the doctor there said he detected a murmur.  However, her regular doctor said he did not hear anything, and it wasn't anything to worry about.  Since then, we have moved around alot, and no other doctor has ever mentioned a murmur.  Also, the doctor today did clear her for cross country, even with that result.  That must mean something good, right??

[NickTurner]

It is crucial that you get this correctly diagnosed.  Long QT is the most frequent cause of sudden death in otherwise healthy (frequently sporty) adolescents.  A repeat ECG with an electrophysiologist or cardiologist can eliminate any source of worry. If long QT is confirmed by an expert, a defibrillator can be implanted.  My own daughter was picked up on a machine as query long-QT. This was infact diagnosed as a partial right bundle branch block (common and benign) which renders the machine based measurements inacurate. Nevertheless long QT is potentially very serious and needs to be eliminated or confirmed. Also there are several other potential diagnoses such as Brugada syndrome which are equally life threatening that can also be simply checked for.  Do this now.

[valger59]

Thank you all so much for the very helpful replies.  I am trying to get through to the doctors office in which she took the EKG yesterday, that I need to get a coply of the EKG and go to a pediatric cardiologist, but there is that red tape that you need to get through first.  I am waiting for a call back, she said she would speak to the doctor.  Yesterday, he said that we have to wait about two weeks for someone to look at the EKG report, and that is of course unacceptable to me, especially now that I have compiled more information on this syndrome.  So, especially with insurance issues, etc...it's so frustrating, but we have to wait until someone calls us and lets us know what to do next.  So, your daugther does not have Long QT?  I am hoping this turns out to be what the doctor said he suspects, that the machine got it wrong.  I don't know how common that is, though.  I suppose there are so many variables.  The thing is, I don't really know if we have a family history or not.  My Dad did die at 69 years old of sudden death, but he had congestive heart failure, due to restrictive cardiomyopathy, that they think was caused by a rare disease called amyloidosis.  I had read where, if it's a family history caused by Long QT, then you would have possibly some sudden deaths or fainting, but in younger individuals.  Also, the only other thing I could think of, is that my older daugther had a sort of fainting spell once, when she was at the track in High School, she is 20 now.  She didn't really go all the way out, but got that thing, where everything looks bright, and she sort of felt like she was going to faint, but didn't really faint all the way.  It was hot, and she hadn't eaten, and once she felt better, we chalked it up to that.  Now, I am of course nervous this could be a sign that our family has Long QT somewhere.  I have frequent heart palpitations, and have been seen by cardiologists, but have not been diagnosed with Long QT, only benign PVC's.  Once, I did have an episode of atrial fib, but I guess this is all not connected.  
    Once again, thank you for the very important information.  I am so concerned that she shouldn't be doing cross country until we get this resolved, but the doctor yesterday, after her EKG, still cleared her for that. So, she wants to do it, and he cleared her, and I don't want to panic her about her health, and not let her do cross country, but we are so scared about it.  She has always been active, and does ballet four times a week, but hasn't been a big runner.  She hikes during the summer, swims, etc., and has never had a problem.  I know that dosen't mean anything, though.  Thanks again, Val

[stevie_wonder]

Hi Val,
I can relate to your concern. It was only a year ago that our family was diagnosed with Long QT Syndrome. We also show borderline Long QT on a resting ECG but have other abnormalities that only an experienced Electrophysiologist can determine.
The United States has many of the world leaders in Long QT Research. My suggestion for you is to contact the SADS (sudden arrythmia death syndrome) foundation and ask for a referral. They are great people and are more than willing to help. www.sad.org is the website.
Your older daughter should also be investigated since she has had a fainting episode. My 20 year old son had a cardiac arrest at the age of 18 as his first symptom. He wasn’t diagnosed then because an inexperienced doctor didn’t know what a Long QT was. Fortunately he was revived by paramedics. It was ruled an asthma attack. It wasn’t until my 10 year old son went for an evaluation after our doctor heard a heart murmur that we discovered the Long QT. His heart murmur is a benign one and is not directly related to Long QT Syndrome, but many of the families I have contact with mention the benign heart murmur as well. Long QT Syndrome is still in its early stages of research and they are learning more and more every day.
In the meantime to be safe, you could stop all activities, but I do understand how you don’t want to make a mountain out of a mole hill. Keep your daughter hydrated and up the salt intake a bit to keep the blood pressure up.  Drinks like Powerade, Gatorade are great because they help keep the electrolytes in balance. Just make sure that her teachers realize that they should call 911 if she should infact faint.
Long QT Syndrome is by no means a death sentence and once treated most people live normal active healthy lives.
Good Luck to you and keep us posted!
Stevie

[valger59]

Thank you so much for the very helpful information!  I took my daughter today to her regular doctor, who is in the same office, to get his opinion.  He first listened for a heart murmur, and said that what he heard were normal variants.  She is very thin, and he said that when you are thin like that, you can hear different sounds, especially from a strong heart.  
    He said basically the same thing as the first doctor, that sometimes it's not an uncommon finding to have the machine print out 'borderline long qt', and then when the pediatric cardiologist actually looks at it, they will declare it normal.  He said something about how the machine interprets things differently for kids.  I guess this kind of thing is hard with kids, unless you are at an expert.  Which is why, even if her results do come back as "normal", I plan on still taking her to a pediatric cardiologist.  You can never be too safe.  
    We feel that we are in a tough spot here, though.  One the one hand, we want to keep her safe, and on the other hand, I don't want to needlessly make her fearful.  Doing either thing can affect her life dramatically.  I don't want to make her obsess about her health her whole life, I do that with myself, and I have a family who does that, so I know that I grew up with that, and it's hard to shake.  That being said, I still believe knowledge is power and it's so important to research everything yourself.  This doctor today also gave her the clearance for Cross Country tomorrow, and so I guess I should feel pretty good about that.  I saw the other doctor on the way out, and he again said that it's not uncommon, and in fact happens all the time, that the diagnosis from the machine will say boderline long qt, and then have it be normal.  Also, I've been having palpitations for years, I think I might have mentioned this, but mine have been mostly PVC's.  I called my cardiologist just to check if I have ever had any indication of Long QT, I mean, I'm sure I would have known, but I just wanted to check, and she called back and said that no, I didn't have any indication of that.  I have had many EKG's with them, an Echo or two, and some other testing.  That should be one good sign, right?  Because, I know my husband could still be the carrier of this, but it's somewhat of a good sign that I don't appear to have it, right?  I know that EKG's don't always pick it up, but I have never fainted or have any symptoms other than the palpitations.  I guess the decision is to let her do cross country tomorrow, and hope that this test comes back normal.  Thanks again so much for all of your help, it's so appreciated!!  Thanks, Val

[NickTurner]

Val, the key issue is not that you have a history of palpatations or health obsessions but that your child has been flagged on ECG as borderline long-QT. In my own case I took my daughter to a pediatric cardiologist because of a fainting spell, possibly related to excersise. Even this expert in structural heart problems could not interpret the machine flagged long-QT interval and referred us to a specialist electrophysiologist, who rulled out long-QT and tested for Brugada syndrome.  I tell you this to illustrate that interpretation of the ECG is a  job for the experts, consider the machine read-out as an early warning system.  In our case, during the interval between the ecg and the tests (about 2 weeks) we were advised to avoid "risky" activities.  I also informed the school that any faint or seeming faint was to be treated very seriously. It is reassuring that you dont have a family history of sudden cardiac death, (your fathers death at 69 is immaterial, as you already have a diagnosis for this), however there is only one way to be a sure.  I would insist on a speedy referal.

[valger59]

Thank you so much for the great responses!  It really makes a difference to have all this information, I can't thank you enough.  I dropped her off at cross country today with her gatorade, and some snacks, and a note for the teacher just in case, just so he knows the situation.  
     Nick, does your daughter actually have long QT?  I wasn't sure if you meant that she had it or didn't.  I am so nervous because I do feel like really what are the chances that the machine was wrong??  It seems like something that might have been picked up, and I'm so grateful that we might have caught something in time, but I hope it's nothing of course.  Today, she really loved her cross country class, and would really be upset to have to quit due to a heart problem.  She has been taking ballet for years, and about a year ago, we found out she had scoliosis, and she has been wearing a back brace for that, and she's just been so brave about it.  It's so hard to think of her having to deal with another health issue.  I thought the scoliosis was bad, this is so much worse!  She should only be wearing the brace at most for another year or less probably.  
     My doctors were reassuring as I said yesterday because of how it was boderline, and they said they see that alot, when it's not.  It makes me mad, though, that they would be giving EKG's to kids and have this happen, they should get a new machine to fit the kids if that truly is a problem.  I can't imagine other parents going through this, which is why I don't think it's as common as they say it is.  They were probably just trying to make me feel better.  I did call for a referral, but of course I heard nothing today, so tomorrow I might be bugging them again.  I don't understand the delays.  I know there are lots of other people with worse problems, but all I have to do is have someone look at it.  
   Thanks for saying that my Dad's death was immaterial, I was worried about that, also I heard that if it's related to QT, then the sudden deaths are at younger ages.  Although, my Dad died a week before he was suppposed to have a heart biopsy at Stanford Medical Center, because they could not figure out the reason for his congestive heart failure, and only suspected amyloidosis, which is a rare thing.  
   I guess my husband or I could be carriers, and not display QT on EKG, but isn't it a rather good sign that both of us have had EKG's and nothing was ever picked up?  I hope that could mean something positive, but I know there are alot of variables.  I have had lots of cardiac tests, and had no indication for Long QT, and my husband has had EKG's I know, and nothing was ever mentioned of Long QT.  Thanks again for all of the help, I really appreciate it!!  It makes such a difference~~Val

[NickTurner]

Val, you should stop looking for surrogates to alleviate your anxiety, I did the same by the way, but it doesn't resolve anything.  My daughter didnt have long-QT, but we only know that from the follow-up.  You should do the same, it is the only way to be sure.  

[stevie_wonder]

I really understand your apprehension in following up on this. I was also told that my son had "ONLY" a borderline Long QT. Well, he also "ONLY" had a cardiac arrest at 18 too. Our resting ECGs had been read by several cardiologists and doctors who said that we were fine and that my son probably just had the acquired kind. But I knew things weren't right and kept searching for a doctor who actually understood Long QT Syndrome. As it turns out, myself and all 3 of my children have it.
I don't mean to frighten you, Long QT Syndrome definitely needs to be treated though. Life does go on. My kids are fortunate in that we have Type 2 and their restrictions are much less and my 10 year old son still runs cross country. Don't let doctors give you a false sense of security about this. Studies have shown that less than 50% of physicians (cardiologists included) can measure a QT interval. In our particular case, it wasn't so much the QT interval as the shape of the T waves that diagnosed it for us. So please go with your intuition and find an experienced Electrophysiologist to read hers and all of your families.
Wishing you the best
Stevie

[valger59]

Wow, I just wrote my whole letter out, and then messed up and didn't post it, so I'll try to write again what I had written before.  Thanks for all the helpful information!  I feel like I'm going through the process of what they kind of make you go through in order to see a specialist.  I am awaiting the call to have me referred to a pediatric cardiologist, and didn't get it today, so I will call tomorrow if I don't hear in the morning.  The process is that they have to have a pediatric cardiologist read the EKG, and then we go from there.  If the cardiologist deems it as 'normal', then they wouldn't need me to go to the cardiologist, but I'm going to do it anyway.  I didn't want to wait for their referral, so I called my cardiologist, and got a name for a pediatric cardiologist and was going to go there, but I'm not really sure which one I should go to.  I am not sure about the Electrophysiologist, since I've never even heard of that, and this is all new to me, but I guess if I go to the cardiologist first, they could refer me to that if I needed it.  Didn't you guys say that a cardiologist would be the first step?  
    I am not going to let the doctors make feel too reassured, but I tend to panic about these things, and I am also trying to hang on to the positives.  I think the good news is that she is symptom-free, and that her Dad and I both have no indication of Long QT on our own EKG's.  I have had several tests, since I have other cardiac issues, and never had an indication of Long QT, and my husband has had EKG's routinely for insurance purposes, and once for hernia surgery that he did, and I assume that if he had any indication of Long QT, they would have said something.  Do they check for that routinely?  Maybe not.  And, I do know that we could be carriers, but it seems from the information that I have gatered so far, that most of the time, when a child is found to have it, other people in the family have it, and usually at least one parent has it.  So, given the current information that I have, I am still extremely worried about this, but feel good so far, if I use my intellect and not get emotional, that so far, it's good news.  Right when the machine printed that out, the doctor reassured me, saying he sees that all the time.  And, the second opinion doctor that I went to said the same thing, that other than a 'normal' result, that is the thing he sees the most, the 'borderline long qt' result.  He says that the pediatric cardiologist needs to read it, and then go from there.  I am hopiing of course it is what they say it is most of the time, just the machine spitting out a result that is more tailored for adults, and also he said the numbers on the machine are very strict.  I have all of this in my head to make me feel better, but am still VERY worried about getting her into a cardiologist.  These things always take time, especially when they don't see the situation as being emergent.  We are traveling this weekend, and I sure wish I knew something more before I go.  She went to cross country today, and ran a mile, and she said she felt fine, only tired.  Which I would expect, since she is not used to running, and it's hot and humid where we live.  Anyway, I feel from boards like this, I'm just gathering as much information as I can, but am still making sure she gets to a cardiologist.  I just hope she dosen't have to quit cross country, she loves it already, and is very excited to be part of this team.  That would be so heartbreaking.  Her usual pediatrician, the one I went to second, said that boderline dosen't put her at higher risk for sudden death.  I know he probably dosen't know this for sure, but still was reassuring.  Also, I did read where borderline prolonged qt is only real long qt syndrome only 5% of the time.  There is that 5%, though.  Thanks again for all the help.  Val

[valger59]

Hi~~
   I wanted to once again thank you all for the information I received during this stressful time, and update you on what's going on.  We got the official reading back from the initial EKG, and it was read as 'normal'.  Her pediatricians office told me that they are read by a pediatric cardiologist.  The nurse confused me, though, when she said that her doctor said that the EKG was normal, and that we didn't need a referral to a cardiologists office, but to watch for any unusual symptoms.  This made me uneasy, and so I tried to ask the nurse if my daughter did indeed have a condition in which we need to "watch for symptoms", but she was vague, and responded in the way most nurses do when pushed on the phone by patients.  She basically said he was just saying that we should watch for those symptoms, as any parent should, but I am thinking that my doctor is just being careful, since she was tagged by the machine as being 'borderline Long QT'.  I should feel rather reassured, though, by the fact that a cardiologist read it, and deemed it normal, but I am still taking her to a pediatric cardilogist to make sure.  We have an appointment in a couple of weeks.  At least that will be a second opinion, and I'm sure they might give her further testing.  She dosen't have any unusual symptoms, nor does anyone in our family.  As far as I know, nobody in my family has Long QT.  I've been tested, and so has my husband.  Anyway, thanks for the help, I just wanted to let you know where we are at with this!  I guess doctors offices can never give you just a black and white answer, it's them sort of covering themselves also.  Thanks~~Val

[keeping it real]

My 13 y/o has been showing signs of what we thought were dehydration, being an athlete and living in Florida. He was eventually sent for an ekg and had a stress test which put him at borderline for long QT. No one in our family has any symptoms, sudden deaths, etc.
The only positive way to know for sure is through genetic testing, which we are waiting on results for now. Just wanted to share that you can find out for sure, why worry when there is testing to clarify the borderline cases.

[wdburn927]

My 4 year old daughter has been telling me that her heart is not beating.  We didn't think too much of it because little ones say silly things until one day I was watching her play & she froze & her eyes kinda bugged out.  I asked her what happened & she said her heart just beat up into her throat.  I called her doctor the next day & she got us in for a echo & an ekg right away.  The echo came back normal but the ekg showed borderline long qt.  They  put her on a holter moniter & we are waiting for the results of that.  Her dad had open heart last year for a congenital defect of the mitral valve but no other problems were mentioned to us.  She herself has never fainted but she tend to be the kid who wears out the fastest.  It was a pediatric cardiologist who read the ekg & recommended a repeat in one month.  I have to say that after I have researched this & her being so small I am really not wanting to wait that long.  Anyway, I would love to hear any thoughts on this.  Thanks so much~Jen

[stevie_wonder]

You could ask your pediatric cardiologist to refer her to an Electrophysiologist (EP). Is there any family history of sudden death or fainting? If there is I would also get yourself and your husband checked out by an EP as well. Cardiologists are famous for underdiagnosing and minimizing Long QT Syndrome. Trust me on that one! Long QT Syndrome is very treatable but if left untreated can be quite devastating. I am glad that I didn't listen to the first 2 cardiologists we saw and sought out an opinion from an EP.
Good Luck!

[wdburn927]

Thanks for the advice.  I spoke with my husbands cardiologist last night & he has given us the name of an EP doc that work with children.  We are working on getting in there.  This is all so very scary!  I hope you & your family are all doing well.  We will make sure to remember you in our prayers.  I know what you mean about minimizing things.  Our pediatrician keeps telling us not to be to overly concerned that worse case is that she'll eventually need a pacemaker.  Worse thing is she could drop dead & we wouldn't have a warning!  There is no family history that I know about of fainting, but we do have a lot of high blood pressure,valve defects & murmurs on my husbands side of the family.  Anyway, thanks for taking time to write!
Jen