Long QT or other arrhythmia

[tazmania799]

My oldest sister died at age 55 due to massive myocardial infarction (documented by autopsy).  

My younger sister died at age 50 and coroner gave cause of death as cardiac hypertrophy.  

Then last year my older sister, age 60, suffered a sudden cardiac arrest on valentine's day.  Her husband is a dentist and immediately did CPR until paramedics arrived.  She was without heartbeat or breathing but after two shocks with defibrillator, her heart started beating again.  She was in a coma for 4 days and then awoke.  They did coronary angiography on day 5 and found very healthy coronary arteries.  On day 6 they implanted an ICD and diagnosis of "Rule Out Long QT Syndrome".  

My youngest sister (age 50) had been experiencing tachycardia on almost daily basis up to 170 beats per minute.  After our older sister's arrest and the possible diagnosis of Long QT Syndrome, my youngest sister went to cardiologist.  They monitored her heart 24 hours a day for 30 days.  She was having "Supraventricular tachycardia" 5-6 times a day.  Insurance company has refused to pay for genetic blood test for Long QT due to her age (usually diagnosed much earlier) and not having a sudden cardiac arrest herself.  My older sister's insurance company has refused to pay for her ICD (done at out of network hospital) leaving her with a $90,000 debt.

So our family will never know if Long QT is the cause of so much early death (in 50's) in our family.  Where does that leave me (age 58)?  I will ceretainly be much more aware of my heart, especially how fast it's beating.  I'm single and live and alone so if I ever suffer sudden cardiac arrest like my older sister, I'm dead.  My internal medicine doctor of 12 years wants me to see a cardiologist.  But it is soooooo rare to diagnose Long QT on ECG.  I have had an episode of documented Long QT on ECG when I was hypokalemic (low potassium).  

After exhaustive research on internet, I found that the genetic blood test for Long QT Syndrome only identifies 70% of Long QT cases.  So you have a 30% chance of getting a negative test and still have the syndrome.  The test is $5,000 for one person.  If the person tests positive (a mutation already known and documented), members of the family can be tested for $900 each.  Getting insurance to pay that $5,000 is the buggaboo.  Until MORE cases of Long QT can be diagnosed by genetic testing, it just doesn't make sense to test knowing the about 1/3 of cases will test negative even if they really do have it.

[tazmania799]

Thanks, I only knew of GeneDx.  Both my sisters remaining living sisters are on Beta Blockers.  I have been on angiotensin II receptor antagonist (Avapro or Benicar) for past 12 years.  I did poorly on Beta Blocker and ACE inhibitors with marked facial and ankle edema.  I also do poorly with any diuretic as my potassium gets very low.

[stevie_wonder]

Hi
Your family history is very suspicious. Insurance companies need to update their information on Long QT syndrome!!
Studies have proven that people who coronary artery disease and Long QT Syndrome have a much higher risk for cardiac arrest and especially in women over 40.
I'm assuming you checked genetic testing out at Familion because those are their prices. There is a new company called GeneDX (www.genedx.com) that has MUCH lower prices. I have had testing done at both places. GeneDX is a more comprehensive test and covers testing that Familion doesn't do yet. Call a rep there and they will be happy to help you get the testing you need.
Testing now covers 75% and more of people with Long QT. They are discovering new genes that are affected and soon hopefully will be 100%.
Since you have shown a prolonged QT on an ECG, I think it is imperative to follow up with an Electrophysiologist, not just a cardiologist.
Beta blockers are the mainstay of treatment for Long QT Syndrome and are very effective in preventing cardiac arrest.
I have an ICD and take beta blockers because of the high risk of sudden death in the women in my family and because my QT interval is extremely long on resting ECGs and during exercise.
Hope this info helps.
Take care,