BRADYCARDIA AND LONG QT

I have been diagnosed with Andersens syndrome a rare form of periodic paralysis that includes a long QT charactoristic and my family is being worked up also. I have a nephew who had his ECG and it showed a pulse rate of 45bpm. He had an echo also which was normal.  He does have periods of lightheadedness, shortness of breath and chest pain.  He does play basketball and considers himself somewhat athletic.  He is 16 years old.  I know through information I received on long QT that long QT can be difficult to diagnose but could this be an indication that he may have it?  I also have bradycardia with a pulse rate in the 40's but had a defibrillator/pacemaker put in this year.  Should my nephew go through other testing to see if his bradycardia is normal?  His doctor gave no explanation for it and left it as "sinus bradycardia" with no follow up.  This worries me.
Thank you for your time.

[Cleveland Clinic, MD]

Dear Laurie,
It worries me as well.  Is there a family history of sudden death?   If yes then I would definately check his QT interval on the ECG and consider him for EP testing and possibly an ICD.  It is not an easy decision and is somewhat of a judgement call so he should be sure to see someone with experience in this area.  A heart rate of 45 can be normal in an athletic individual but he may need additional work-up.  Let us know how things turn out.

[Laurie]

Hello doctor,
Yes, I have a few young deaths in my family on my dads side .  All cardiac related. An uncle age 35, his son age 34 and my great grandfather age 28.  The Andersens comes from my mothers side so I almost wonder if we got hit with two long QT genetic defects.