Long QT syndrome - LQTS Video

Learn about Long QT syndrome - LQTS Andrew D. Blaufox, MD Pediatric Electrophysiology Schneider Children's Hospital . Associate Professor Clinical Pediatrics Albert Einstein College of Medicine. Medical School: Albert Einstein College of Medic...
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Male Speaker: Long QT, what is that? Andrew D. Blaufox: The Long QT Syndrome is a inheritable problem for the most part, although there are acquired forms of Long QT Syndrome. What that involves is that it takes a longer time for the heart to reset itself. The heart has a beginning phase where the charges get propagated down through the tissue and then it has to reset itself, and in the Long QT Syndrome the resetting takes longer and incurs variable times throughout the heart muscle. This sets patients up for having malignant, dangerous arrhythmia such as torsade de pointes and ventricular fibrillation and puts them at risk for a sudden death. The problem with Congenital Long QT Syndrome occurs in either influx of sodium into the cell or efflux of potassium out of the MAO sites, and then prolong the actual potential duration which then gets manifests as this measured prolong QT interval. Male Speaker: Just looking at a person, listening to a person, could you pick up Long QT without a cardiograph? Andrew D. Blaufox: No. You can have suspicions that someone might have Congenital Long QT syndrome if they had Syncope, let's say, with or without exercise, without any prodrome of dizziness that's more typical of a simple faint. Let's say they had a family history of a close degree relative who died suddenly with a startle or something in that nature, then you might be highly suspicious that something like the Long QT Syndrome -- Male Speaker: So QT could run in families? Andrew D. Blaufox: Long QT Syndrome, its a genetic problem, so by and large it runs in families, although spontaneous mutations do occur, so you may have isolated cases, but by and large, if you find it in one family member, you need to look at all the other close relatives.

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