SADS Foundation :: Sudden Arrhythmia Death Syndromes Foundation

In 2003, I had sudden cardiac death. I was brought back with CPR, no shocks. I was admitted in the hospital by some miracle because the ER doctor I saw said it was a seizure and that they don't usually investigate the first episode. He did some blood test and found out my electrolytes were all really low. My EKG showed prolongued QT that this doctor didn't pick up..another doctor saw it and took an interest to it. I had an echocardiogram some days afterwards, I had shortness of breath, increased urination, etc) that found my left ventricule to be severy enlarged and an ejection fraction of 30%.. They treated that, not very optimistically. I went on to have a stroke so they kept me in the hospital for rehabilitation for a month. My cardiologist kept monitoring my QT, even during my PT and released me from the hospital 31 days later when it went down to normal values..
My heart size went to normal again and regained heart function (55%, a bit lower than normal), my QT was normal for about 4 months when it became long again. I have no family history of sudden death, only an older brother that died in a car accident.
Now the question is: did I have long QT before the cardiomyopathy (that was suspected post partum..I had a 4 month old child at the time), or is the long QT a side effect of it?. As a child I tired easier than other kids my age. I'd play tennis with my mom who smoked 2 packs a day, had 4 kids and a job and was 25 years older than me and get super tired way before she did.
I have 2 kids (8 and 5) that had the epinephrine test + holters without any sign for concern.
Until now my cardiologist said we could assume the LQT showed up after my heart failure but he's been pushing for genetic testing lately.
I take 50mg toprolxl and have an ICD that has never fired (knock on wood).
They'll do the testing on me, and we'll go from there. My mom has a borderline QT , but normal during a stress test.
My husband was kind of hard to convince for the genetic testing. Because of insurance and cost (BCBS) and out of network thing..you know?. But as off today we had PGX health start my test.
I want to thank all of you that shared your stories in this board, for pouring your hearts out to help other people. Some of those people you helped, or your loved one helped may even be my kids..Thanks again and I'm really sorry for your pain and losses.
God bless,
Dolores

It sounds like you have had quite a time of it and I'm glad that you are doing well now. The short answer to your question is that LQTS can be part of any cardiomyopathy as anything that affects your heart muscle also effects the way electricity is conducted through your heart. What this means is that when your QT interval is long you can have a sudden cardiac arrest just like anyone with familial LQTS.

However, it's possible that you also have familial LQTS and developed cardiomyopathy separately. In that case your genetic test would be positive (if you are in the 75% known mutations) and your children, siblings, etc. can get a genetic test done for your "family" gene.

Hope this helps and let us know how things turn out.

Hi, an update: as it turns out I do have familial LQT, a mutation has been identified: the report says *the tests indicate the presence of what is consistent with LQT11, QT1 variation. Thus this with your history would confirm the familial presence of this potassium channel defect*.
Do you know anything about LQT11, and what does QT1 variation refers to?
I'm still waiting to hear from my children EP..They both had 48 hrs Holters and EKG's and epinephrine stress tests 4 years ago. They were on the clear but I do not know if the fact that my mutation has been identified changes that, or what's next for them.
My family doesn't want to hear about it...I just wish they did so we can try and keep the kiddies safe..Both my parents and siblings had normal EKG's 4 years ago too.
What would be a way to go around this? Testing my parents first and going down from there?. I suppose they'll be the easiest ones to convince for the genetic testing..
I'll be seeing my EP in a couple of months..
Thanks!