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My daughter (aged 7) has just been diagnosed with CPVT. I would like to speak to anyone who has experience of the condition, so that I can learn more about it and to understand what limitations I need to make on her lifestyle. Can anyone help?
Exercise Surgery Sudden cardiac arrest Atenolol Propranolol Syncope Beta blockers Memory Stress
Hi,
I was diagnosed with CPVT at the age of 37 - six years ago. It is, I am sure you are discovering, a very rare condition, or at least survivors are very rare. It is a great thing that your daughter has been diagnosed so young - the majority of people with CPVT don't live out of their teens because they have not been diagnosed and treated.
I am cared for in the UK by a professor in cardiac electrophysiology and the following is what I have been told by him.
Initially the treatment is betablockers, and if these are tolerated then further treatment may not be needed - other than lifelong betablockers and monitoring. The downside to these drugs is fatigue, which can be unpredictable (i.e. not related to activity) and low blood pressure which can result in fainting. The usual advice is to eat regularly, drink plenty of fluids and rest when necessary.
If betablockers are not successful then an ICD (implantable cardiac defibrillator) may be offered. This is the safest option as it means that any potentially fatal arrythmia will be dealt with by the ICD and sudden death due to CPVT is highly unlikely. However, ICDs can be difficult to cope with as they are sometimes 'unpredictable', require replacement regularly and can mean limitations in activity. There are also psychological consequences of having an ICD as well as having a sudden death syndrome.
I have an ICD as betablockers dropped my heart rate too low. Its taken a while for me to adjust, but maybe children adjust more quickly.
The other advice that I have been given is to avoid swimming (its a very high risk activity for CPVT), avoid extremes of exercise or stress(no competitive athletics, cycling, mountain climbing, etc), no alcohol, no chocolate and no caffeine. I would imagine the chocolate thing could be tough for a child, but as I have an ICD I do sometimes have a piece even though it triggers some arrythmia - as does fatigue, exercise or stress (arguments, upsets etc).
You may also find the following forum useful as there are a number of parents with children with similar conditions who regularly post. http://www.icdsupportgroup.org/board/
Hope this helps and best wishes
Karen
BTW,
The other reason swimming is a definate no is the risk of drowning with a loss of consciousness from an abnormal rhythm - whether the person has an ICD or not.
Karen
Hi,
It is suspected that my daughter has CPVT. Dr. Michael Ackerman at the Mayo Clinic in Minnesota reviewed her records and felt strongly that this is what she has. She was originally diagnosed with LQTS but when we had genetic testing done it came back negative. We had genetic testing done for CPVT too and this also came back negative. This does not necessarily mean that she does not have it though. When Dr. Ackerman discussed the possibility of her having CPVT, he said that she should not only be on beta blockers but also have an ICD as CPVT is more aggressive than LQTS according to him. She is taking the medication and has an ICD which has shocked her during three separate events in the past four years. She was on Atenolol when she had her events but was switched over to Propranolol and the dose was increased. We have not had any problems in over a year. She is not allowed to swim and is restricted from competitive or strenuous activities. I would suggest that you get in touch with Dr. Ackerman as we have been very impressed with him and he has been very willing to answer all our questions. He has an email that can be found on the Mayo Clinic site. I am glad that your daughter was diagnosed early. I wish you all the best.
Karen
Hi Karen
Thanks for your reply. Could you possibly send me or post Dr Ackerman's details as I can't see them on the website. I would also love to talk to you in more detail about living with CPVT. Please contact me if you would like to.
Charles
Our 15-year old has CPVT also. He had a cardiac arrest at school in March and was resusitated with an on-site AED that saved his life. He has an ICD plus two types of meds (a calcium channel blocker since CPVT is essentially a calcium problem) and beta-blockers (nadolol). He is using a combination because of the side effects of the beta blockers (mild memory issues, fatigue). He is being treated at UNC and Duke (with consultation with an expert in Italy) and just underwent another surgery to change one of the ICD leads (was not reading his heart correctly).
We have been told that he will be able to engage in most activities (except contact sports) but should not swim alone ever. He has already started playing tennis again and we are hopeful that he will be able to be fully active in the future. He has no dietary restrictions but I want to ask his doctor about the chocolate, etc.
For us, the most difficult issue has been the ICD and its' technical issues (e.g., over-counting, under-counting, fear of inappropriate shocks - he had one). Another difficult issue for us has been the effects of the beta-blockers on his memory and school work. While subtle, the effects have been very noticeable to him and we worry that the doseage is affecting his quality of life. We are hoping that the dosage will be decreased with the addition of the calcium channel blockers.
Good luck,
Christi
Our family has also been tested (genetic testing) but only our son has tested positive for CPVT. Did you daughter go through genetic testing?
Hiya, I have CPVT and an ICD.
Bascially I was DNA tested for Long Q T but this wasn't present so they 'put my problems down to' CPVT.
I have a 3 year old daughter and although she is showing no signs I would like her checked but the NHS don't seem to want to do this unless she shows symptoms.
Is there anywhere else I can go to so that I can have both my diagnosis confimed and her heart checked?
Would the Mayo clinic do this? I am 34 and have had syncope since I was 13.
Hi, Our 7 yr old son had sudden cardiac arrest on 3/28/08. He was sleeping and we went to wake him and found him. Our burgler alarm had gone off(I forgot to turn it off in the AM) and we know he was down for 12 min before and CPR. He was put in hypothermia and had AICD put in. He was found to have LQTS QTc now is 491 was 598 after arrest. He is doing ok, but we got our Familion LQTS test back and it was negative (2 polymorphism found on 2 known LQT sites. #3 and #5). Dr Moss and Dr Daubert in Rochester want us tested for CPVT currently waiting for insurance approval. They did mention some other genetic research for other LQT sites that they want him to be in. One of them is at Mayo Clinic. We dont have to go there just send the blood and all his medical records. Is this Dr Ackerman somone you think would be beneficial to talk too? We love our doctors and have the leader of LQTS right in our back yard but it never hurts to get newer information and technology. Thanks
I've had cpvt for 5 years that I know of. I'm sorry your daughter has it.
you can e-mil me at harvest@execulink.com if you like and then I can give you info on how I have handled having it
Hi Christi
We have been undergoing many stress tests, holters, 2 ep studies,etc. Over the past summer after another failed ep study our cardiologist decided to perform a stress test himself and to let our 9 year old son continue with the test once he showed the abnormal arrythimia. During which he noticed a particular arryhthmia that looked the kind that would be cpvt. We had the genetic test done and as of today we just got the results- positive!
In a strange way we feel relieved to know what he finally has but in the same breath scared to death. All of this came about March of 2007 when a parent found our baby Brandon laying in the school hall unconsious.
This year since school started he has started fourth grade and what you wrote about your child really caught my attention. Brandon has always got high A's in school. This year his reading and writing have dropped to 62 and 53 and they both involve reading and then anwering questions based on what they have read. I have not been able to get my head around his grades dropping from placing third place out of the whole third grade last year to this! that was until I read what you wrote. Is that true about the Nadolol? I have not read anything about that until now? I would appreciate any info. We meet with the doctor on monday and i will definitely be bringing this up? Hope your son is doing good. Bye for now and look forward to hearing from you.
Thanks Sarah.
Hello,
My son who is now 23 was diagnosed with Long QT when he was 12 - after 3 fainting spells and no diagnosis by his doctors. He was put on Beta Blockers and it was recommended to us to implant an ICD. It was hard for us to consider cutting him open and putting in and ICD so we decided to wait.
The Beta Blockers seemed to be working fine - he did have 2 more fainting spells (one while swimming at camp and his brother pulled him out!). When he was 16 we had a family party at home and he fainted while dancing - and stopped breathing. We performed CPR and called EMT. When they arrived many minutes later, they shocked his heart back into rhythm, but he didn't wake up and was seizing. We were in the ICU for 5 days and then had a Defibrillator/Pacemaker implanted. He has since been a participant in the Mayo Clinic/Dr. Akerman's genetic study and has been diagnosed with CPVT. He is very athletic, plays softball on a social team, ultimate Frisbee, skiis, works out, even runs sometimes - and I feel safe about it because he has his ICD. IF anything would happen - he is covered. If he didn't have and ICD, I don't think he wouldn't be doing all of these things - wouldn't be living his life to the fullest. Of course he has taken a Beta Blocker since he was 12, but you just never know when that big adrenaline rush can happen - car crash? nightime/dark scare?
What I'd like to impress upon you - is that it is hard to think about implanting a device in your child - especially a small child - but you don't ever want to go through what we went through. We almost lost him - the doctors couldn't believe that he wasn't brain damaged from the lack of oxygen. We were lucky - don't rely on luck. He has had his ICD replaced once - it is really no big deal and, as he has grown into a young man, it hardly even shows anymore. We all just feel so lucky to have had the second chance to get the ICD and have him "carry around" his own life saving device. He feels safer too.
So sorry to hear of your ordeal - our son went down too at age 16, but is doing great at age 23 now! :)
We have been in the Ackerman study for many year and they just found our son's specific mutation - and rediagnosed him from Long QT to CPVT. It really didn't change his day to day at all -same meds. But, it did allow the rest of the family to send in blood samples to be tested for the same gene - for free! And, since we know the exact gene, our son can have his children tested when they are born. It takes awhile to get the results, but it is worth it. And, Dr. Ackerman's assistant, Carla, is great to talk to. Hope that helped!
Hi - We have been in the Ackerman genetic study for several years and they have just identified my son's specific gene... so they asked for the rest of the family's blood to test. We sent it in and are awaiting the report. I would definitely contact them and ask - (502) 284-0101 (you want Carla in the Research Dept.). Good luck!
Hi to whomever Mars is. My name is Sarah. Could you please tell me more about the Dr Ackerman study. I know you left the details ( phone number and name). How did you hear about it. We are meeting on Monday over brandon and also having to bring our youngest with us to start EKG on him. We have been told the younger son Dylan will be tested genetically next then us. Our insurance is refusing to cover the test and i was under the impression that famillion pgx health were the only ones that performed the test. We are already in to it for $3,500.00 for the test and each additional member will be $900. (not that we wouldn't pay it because we are happy to get the answers we need but obviously if we can save the money we would like to.
I really appreciate any info you can float my way. my personal email is latteda@hotmail.com.
So happy to hear your son is doing okay.
Thankyou and all the best
Sarah.
Hey Everyone,
I'm glad I found this site. My mom was diagnosed with CPVT in July after being tested along with all of her brothers and sisters. Two of my cousins, my mom's nephews, died suddenly a couple years back and since then we've discovered that our family carries the gene mutation for CPVT. I was tested in August and found out yesterday that I also have CPVT. I will be seeing a Cardiologist in November but until then, does anyone out there have any suggestions for me? I was told not to do much hard physical activity and to get a medic alert braclet. What are your suggestions?
Hey,
I am eighteen now, and I was diagnosed with CPVT a little over a year ago. I played softball since I was five, and on May 10, 2008 when I was sixteen (almost seventeen), I fell to the ground at one of my softball games and went into sudden cardiac arrest. CPR was performed on me, and then I was resuscitated with an AED (after being officially dead for a minute). I was in the hospital for two weeks, and they diagnosed me with CPVT and they implanted an AICD. They told me I could never play softball again, at least never competitively. I was crushed. They told me to be careful when exercising, to not let me heart rate increase too rapidly. There are many things like this that people have already mentioned above. She should definitely decrease her caffeine intake, because if you have too much in your system you can definitely start to feel the arrhythmias, and it is very unsettling. My doctors told me that my defibrillator would probably never have to shock me since then it has shocked me twice and paced me three times. We had problems with the ICD incorrectly reading my heart rate so last November I had to have a second surgery which was the addition of a second lead to better read my heart rate. Since then I haven't had any major problems, besides some aches and pains every now and then. Aches are common, with the muscles growing around the ICD like a pocket, every so often little movements may make it hurt. I will also say that when it is cold outside it also makes my chest ache a little more. I hope I could be of some help. If you would like to know more, just please contact me.
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