short QT syndrome 1
Definition A heart disorder (OMIM:609620) characterised by idiopathic persistently and uniformly short QT interval on EKG in absence of structural disease, resulting in syncope and sudden death.
Molecular pathology Defects of KCNH2, which encodes the subunit of voltage-gated, potassium channel cause
• Long QT syndrome 2
• Short QT syndrome 1.