anyone with experience of long QT syndrome or similar (cardio)?

[mummypig]

We paid for a private ECG to check if this was the cause of ds2's seizures and it showed prolonged QTc. So I am now going back to the GPs to ask (for the third time) if they could refer him to a paediatric cardiologist. I feel that this potentially very serious cardiac condition is far more scary than him having epilepsy, so if anyone has been in this situation I would appreciate any words of wisdom. Thanks

mummypig - hi.

My dd has significant heart problems - one of which (a coronary artery anomaly) is related to sudden death. I know how worried you are. The charity Cardiac Risk in the Young has some good information and can offer support. (the site appears to be down at the moment though).

How old is ds and does ds have an epilepsy diagnosis or are they spells relating to Long QT? Your GP can refer as an urgent case and get a really quick appointment if he feels it necessary. Which area do you live in?

hi thanks for the answer, and sorry to hear about your dd. How old is she? How do you both, and other family members, cope with the implications of her condition?

I am hoping that this is just a scare, but if necessary I suppose I will have to come to terms with the risk of sudden death. It's quite something to have to think about. Ds2 did have heart failure at about 6 months but at the time it appeared just to be pericarditis, and we were assured he had no congenital heart problems. Nothing showed up on the echos or ECGs in any case - but from what I understand, Long QT Syndrome might not show up at that point anyway.

Ds2 is 3 1/2 now and yes he does have an epilepsy diagnosis. Also, reassuringly I suppose, his EEG (taken about an hour after one of his seizures) showed epileptiform activity. So it's quite possible that the epilepsy diagnosis is correct, and the only reason for the seizures.

However, a cardiologist friend of ours expressed concern that although the anti-epileptic drugs seemed to have controlled his tonic-clonic seizures, they weren't stopping the falls (which happen several times a day) and that cardiac problems hadn't been ruled out. We saw a paediatric neurologist privately and she requested an MRI and an ECG just as a matter of course, not expecting anything to show up. Now the ECG shows the prolonged QTc and both our neuro, and the cardio who interpreted it, think we need a referral.

I suppose I have lots of questions - like whether the QTc measurement inevitably mean he has a problem, and if so, whether it is likely to be genetic or acquired (as I've read that long QT syndrome could be either). Would the CRY helpline be good? When I looked at their website it seemed to be more geared to helping families where there had already been a sudden death.

We live in Surrey and our cardio friend has a colleague at GOSH who specialises in paediatric electrocardiology and says he can see us within a week. It's just getting the referral that has been a problem. I'm hoping the GPs will take notice now that i actually have the ECG. We can't carry on paying for private healthcare as we have no insurance. Plus I do feel that the NHS doctors 'caring' for ds2, underpaid and overworked though they are, should have at least tried to rule out this possibility themselves, given how potentially serious it is.

hello
my daughter 2/10 (a twin) was having seizure type events from 16 months she would collapse turn very blue and heart would stop but restart within 30-40 seconds, i am a paediatric nurse and at first quite scared but eventually i wrote the referral myself and handed it to my GP and said refer as a urgent case luckily my daughter has a condition called Reflex Asystolic Syncope which basically means she collapses in moments of stress or fear! She was tested for cardiac diseases etc.. as urgent as if a child is suspected of having cardiac issues then this has to be treated as urgent! Listen I know you went private for tests but your GP has a duty of care to you and your child and GOSH is the best place to be seen, Take a deep breath go to the GP and request an urgent referral if it is a NO then please get in touch with your local health authority or PCT and tell them your situation YOu and your child have a right to diagnosis as a long QT can be quite a serious thing if that is what it is!! My paediatric consultant who i saw within 10 days of seeing my GP had us in hospital for cardiac tests quite quickley to rule OUT long QT as urgent!! Please try to see the people who know and that is GOSH.
Good Luck its a nightmare I know both personally and professionally!!!!

I can't help you or really offer any advise but I just wanted to encourage you to go back and pester, pester, pester! I really don't want to frighten you but I know a family for whom Long QT Syndrome had very tragic circumstances. Please get this ruled out, it can be an extremely dangerous condition and I would hate anyone else to have to go through trauma because a dr can't be arsed to check properly for something.

hi - mummypig. It's good that ds has already had an Echo. I am aware that there are structural heart defects that can cause 'spells' but that would have been ruled out by Echo. I don't know much about Long QT TBH but I wonder if it's possible that it was caused by ds2's pericarditis(?) if that's what it was. The helpline is worth a call I think, and I hope you can get your referral and get seen asap, to put your mind at rest at least.

dd is 14 now ad we do OK I have my moments but she is well aware of her limitations, very 'in touch' with how she feels and acts accordingly.

Let me know how you get on.

take a look at www.sads.org.uk

Kangaboy has been investigated for Long QT and so please push push push for a DX

Good luck

hi all, thanks a lot for your messages. I am very and that this could be terribly serious and the GPs just don't seem to be dealing with us very well. Thanks matso for suggesting getting in contact with the PCT as a next step. I will start off being friendly and reasonable tomorrow but if they won't budge I will suggest that and see if it will get them moving. Our cardio friend has also said to get the GP to phone him directly if I don't think the appointment is going our way. It's crazy - he has already written them a letter explaining what he thinks and saying they really should do a referral, and they still refused...

I will keep you posted on our progress. Thanks again, everyone, for your sympathetic and helpful messages.

well the gp I saw today said she would try to sort out the referral but as I suspected, it's funding from the PCT that is the problem... I am waiting to hear more at lunchtime

What? Funding for an urgent referral? I'm gobsmacked TBH mummypig. Why is it a funding issue?

I can't pretend to know about how the PCT is run, but the GP seemed to have to wait until someone from the PCT approved what she was doing, before she could fax the referral over. Anyway luckily it was all approved and the referral should be being faxed as I type, marked urgent. Believe me, I would have been making a huge fuss if it hadn't been approved. But the piggies are relatively happy for now...

Hallelujah! Hope you get an appointment through quickly. Keep us posted.

Hooray . Ds2 has an appt at GOSH next week to have tests and see the consultant. They called this morning and I don't have the letter yet, so not sure exactly which tests they are doing, but at least something is happening.

Hi muumypig, really pleased that you have an appointment, I had long QT caused by wolff-parkinson-whyte syndrome from being a baby, but only became symptomatic when I was 14. Finally got treatment when I was 19 but it was a bit of a scary time.

The earlier it's detected the easier it is to treat and the shorter the procedure. Also the treatment I recieved has come on in leaps and bounds in the last 20 years and is far more sophisticated. When I had the procedure (1994) it took 8 hours!! Now apparently it only takes half an hour or so for a baby/toddler.

I've now been asymptomatic for 14 years!! Best of luck to you and your dc xx

deffo brill news

A tip from me would be to get there early cos if it anything like at the Brompton you check in and then go for the ECG and then come back again to consultant {at the time of your appointment} with your ECG print out

It may be different @ GOSH of course

Good news!

Test will probably be repeat ECG, possible ECHO, exercise test, 24 hour or 7-day holter monitor.

peacelilly do you have a pacemaker?

mummypig - does ds take meds for his seizures? Some meds can be a cause of drug induced LongQT you know.

List here (American)

No, no pacemaker, there was a risk that during the procedure one MAY have to be inserted and I had to sign a form consenting to it, but luckily it wasn't neccessary

I took all the tests as mentioned below too.

That is good news that you managed without one

hiya i know this probably isnt helpful, but my best mate has long QT and has a very very normal life, job everything just thought that might reassure you theres some good stories

all the best

watch ITV1 in a few minutes they are at GOSH

at GOSH in a mo

Oh thanks for posting RTKM, but I missed the programme (out shopping with ds2 ).

Thanks all, for your encouraging words. We have been told tests are at 10.30, appt with consultant at 11.30 so hopefully we won't have to arrive too early? He had a few echos when he was younger so I'm not sure if they will need to repeat them although I suppose it's better to be careful. I hope they do a holter at some point as then it will pick up all the falls he's been having. The last ECG was just a resting one, and very brief.

Great to hear from peacelily and sparkly that LQTS is not necessarily as scary as I originally thought.

Yes, saggar, I think it's highly likely that his prolonged QT was acquired due to drugs, but not the anti-epileptic ones. He was on domperidone between about 6 mo and 2 yo due to severe reflux, and that is on the contraindicated list if you have LQTS as it is known to increase the QT interval. It wasn't one of the side-effects I was warned about... domperidone is very much promoted as the 'safe' alternative to metaclopramide and cisapride. At the time I remember reading about all the possible neurological effects of having those other drugs and thinking 'we can do without that'. His current AEDs aren't on the contraindicated list so afaik don't have the effect of prolonging the QT interval. Anyway, I'll let the GOSH people know his history and they will be able to draw conclusions better than all my hypothesising.

Bye for now

That is good that they have given an appointment time for tests

Everytime DS goes he has an ECG so I think they will repeat the echoes with their own equipment so they can see the results with their own eyes iyswim

DS had a 48 hour holter and he may be having a 7 or 14 days one next time and he was able to do everything with it on ie the stickies are very stickey

hi just updating, and a quick question (although I can call GOSH about it if necessary). Ds2's appt at GOSH was yesterday - we had to postpone it as we all had a nasty virus for a while. The ECG and echo results were all good, and the consultant said if he hadn't seen the earlier ECG he would have said ds2 was fine. It seems unlikely he has long QT syndrome, but since this can be variable and take a while to show up, they will still check up on him in about 6 months and possibly for longer.

Just wondered - we have taken the wires off ds2 but what should I do to switch off the 24 h holter? Dp tried pressing both the buttons and they just said 'patient event'. Do I have to take out the card or shall I just leave it?

I know I can call GOSH to check if necessary, just thought someone on here might be able to tell me without bothering them!