It's not really associated with a pause in heartbeat. In fact, I'm not sure that someone with
long qt would ever know it without having someone do an
EKG and diagnosing it, or them having severe arrhythmia symptoms like
syncope due to Torsades de Pointes or some other ventricular arrhythmia.
I think the main concern with
long qt is the heart is taking too long to repolarize. How that works is this - the heart first depolarizes (fancy term for a discharge of electrical current that makes the muscles in the heart to contract), and then it needs to recover and make itself ready to contract again by "recharging" itself (that's not exactly what happens, but it's good enough for this explanation). With
long qt, the recharging process take longer than it should. The reason this is bad is if the person also has
PVCs, during the extended time the ventricles are taking to recharge, an ectopic focus fires off an electrical charge causing a
PVC when most of the heart cells aren't recharged and ready to contract yet. So some of the cells contract, other ones don't, and little or no blood is pushed out of the heart because of the lack of synchronization in the heart cells.
Now that's worst case scenario. WORST CASE. Many people have mild
long qt and never have a problem.
And without
long qt,
PVCs really aren't a threat.
As for the test - Wikipedia has a decent layout of how the risk is scored.
Long QT syndrome - Wikipedia, the free encyclopedia
Hope that helps.