Demystifying the Congenital Long QT Diagnosis.

The incidence of the congenital long QT syndrome is about 1 in 2500 births. This is likely an underestimate as sudden death with a normal autopsy may be the first and only presentation. There are 12 genes responsible for 3 main types of c-LQTS. The presentation is one of aborted sudden death or brief arrhythmia. This usually manifests as sudden, without warning syncope, near syncope or prolonged syncope with seizures. Many are misdiagnosed with seizure disorder or vasovagal syncope.