Bradicardia with REVEAL

[macca61]

Hi all, with a family history of sudden death, my 15 year old son has had  2 blackouts ECG shows early repolarisation in all leads, bradicardia 40-50  he is not at all athletic. He had a drugs challenge which ruled out Brugada, he has now an implanted REVEAL recorder which will be 'read'  in November.  He  tires easily  and recently he has had a spiked pulse  120+ , also the least thing  a cold or a tummy upset he is wiped out.  Has anyone else experienced something like this???  I would appreciate any input. Thanks.

[grendslori]

Hello again. My question is: did your son ever have an EP Study done by an EP Specialist? This is VERY mportant. Why are they considering a Defibrillator instead of a Pacemaker? A defibrillator is used to shock the heart out of an arrhythmia and the pacemaker is used to keep the heart beat from falling too low. Thank you for asking about my daughter. She was diagnosed at the age of 6 and was transplanted at the age of 22. She is now 35 and they are starting to evaluate her for a second transplant. She has had a very hard life and this heart disease has robbed her of so much. The Forum I was speaking of is the Pediatric Cardiology Forum; I was not sure if you were writing on the regular Pediatric Forum or the Cardiology Forum, neither being the Expert Forum. Take care

[macca61]

Hi, apart from obvious Early Repolarisation, his heart is normal sinus rhythm but bradycardic, all the routine cardio investigations have all come back  normal, His Dr has initially said he would have a defibrillator, but  after discussion with other cardios, it was decided to implant the  monitor first. the first reading will be in 4 weeks time. The pediatric forum has been informative, I didn't think to look there , he has only just turned 15 but quite manly,  how old is your daughter??  did she have a transplant??
Regards

[grendslori]

Hello, it is VERY important for your son to be evaluated at a facility who sees a lot of children and teens like him. Bradycardia with rates in the 30s range can cause the heart to stop beating which obviously can lead to a sudden death event. (my own daughter had rates like that and we were told this by her cardiologists in Houston). Your sons both should have EP Studies done to look at how their heart's electrical systems are working and to find out exactly why this is happening. Reading your post was too familiar to me; my daughter had a very severe form of Bi-Ventricular Hypertrophic Cardiomyopathy with a whole slew of electrical issues (WPW,SSS,LBBB,RBBB,A-Fib and a Maheim Fiber) probably caused by a very rare form of Glycogen Storage Disease (PRKAG2 gene). You need to have genetic testing done on your sons especially in light of what is happening in your family. I would not be sitting around waiting to find out the results of a loop monitor if it were my daughter, especially with heart raytes that low. Get them to a University Hospital where transplants are done. (NOT that they need a transplant; it's just that the facilities are so much more advanced than a regular hospital and they see more kids with these types of issues) Take care.  (On a side note, we also have a Pediatric cardiology Forum here oin MedHelp for the support of families; this is NOT the Expert Forum)  

[spade22]

I'm going to PM you some information so check your in box. :-)

[macca61]

Hello again,  he was implanted in July and my concern is to wait until Nov to see the results, meanwhile he is still having bradycardic episodes interfering with his life.
His older brother has had the  same drugs challenge  he is also Brady and same  repolarisation ECG,  but no symptoms. Another brother  will be investigated in the near  future,  We have all been tested genetically but  no results of that yet.
It is worrisome to wait another month for results of the monitor, but I will contact SADS  to see if they have heard of similar experiences. Thanks for your replys. Hope all is well with you. Regards.

[spade22]

Just reread your initial question and see there is a history of sudden death in your family.

Based on this history, it's possible that your son has a genetic mutation which is causing his symptoms (and those of other family members) - this is likely why he was tested for brugada syndrome,  

I strongly encourage you to contact the SADS foundation and explain your sons history along with history of sudden death in our family. The SADS foundation can help and will be able to point you in the right direction and will provide  recommendations for specialist EPs that will be able to help properly diagnose your son.  Certain medical conditions are quite rare and are very difficult to diagnose and it's important that your son sees someone who is an expert with SADS conditions (based on your family history, this has to be "considered" - but it doesn't mean he has a SADS condition )

[spade22]

Based on these numbers, this cant be what I was thinking. Here is a link in which a similar  question was asked and might be useful:

*************.****

Hopefully the recorder will provide some more insight when it is read next month.  From what i have read, early repolarization is typically benign. Has your sons EP discussed potential treatment options fro the bradycardia?  If he has bradycardia with symptoms, then a pacemaker "might" be one of the treatment option that will be considered.  

[macca61]

Hi  last    2  ECG's   HR  38   QTc 398    and  HR 45  QTc  346

[spade22]

Do you know what your sons QTc value is?