long QT syndrome type 4 (sick sinus syndrome with bradycardia)

Genetics

• associated with defects in ankyrin 2 (ANK2)

Clinical manifestations

• displays many atypical features compared to classical long QT syndromes

Diagnostic procedures

• electrocardiogram
  • pronounced sinus bradycardia
  • polyphasic T waves
  • atrial fibrillation
• cardiac repolarization defects may be not as severe as in classical LQT syndromes
• prolonged QT interval on EKG is not a consistent feature

More general terms

• long QT syndrome

Additional terms

• sick sinus syndrome; atrial fibrillation with bradyarrhythmia; tachycardia-bradycardia syndrome

Database

• OMIM: https://mirror.omim.org/entry/600919