long QT syndrome type 10

Genetics

• associated with defects in SCN4B

Clinical manifestations

• syncope & sudden death in response to excercise or emotional stress
• may present as sudden cardiac death in infancy

Diagnostic procedures

• electrocardiogram:
  • prolonged QT interval
  • polymorphic ventricular arrhythmias

More general terms

• long QT syndrome

Database

• OMIM: https://mirror.omim.org/entry/611819