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Disease characteristics

Excerpted from the GeneReview: Romano-Ward Syndrome
Romano-Ward syndrome (RWS) is purely a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the ECG and the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with RWS. Syncope typically occurs during exercise and high emotions, less frequently at rest or during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of individuals with a disease-causing mutation in one of the genes associated with RWS have symptoms, usually one to a few syncopal spells. While cardiac events may occur from infancy through middle age, they are most common from the pre-teen years through the 20s.

Associated genes

  • Also known as: ATFB1, ATFB3, JLNS1, KCNA8, KCNA9, KVLQT1, Kv1.9, Kv7.1, LQT, LQT1, RWS, SQT2, WRS, KCNQ1
    Summary: potassium voltage-gated channel, KQT-like subfamily, member 1

Clinical features

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  • Torsade de pointes
  • Sudden cardiac death
  • Ventricular fibrillation
  • Abnormality of the ear
  • Syncope
  • Prolonged QT interval
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