The Twiddling Andersen

Tex Heart Inst J. 2009;36(4):349-51.


Andersen-Tawil syndrome is an autosomal dominant condition characterized by dysmorphic features, periodic paralysis, and ventricular arrhythmias. Twiddler syndrome is characterized by intentional or inadvertent manipulation of implanted devices in the pacemaker pocket. We describe an unusual case of an 8-year-old girl who had both syndromes.

Keywords: Andersen syndrome/genetics; KCNJ2 protein, human; Twiddler syndrome; cardiac complexes, premature; defibrillators, implantable/adverse effects; electrodes, implanted/adverse effects; foreign-body migration; long-QT syndrome/genetics; mandibulofacial dysostosis; potassium channels, inwardly rectifying/genetics; self-injurious behavior/complications; syncope; tachycardia, ventricular/genetics.

Publication types

  • Case Reports

MeSH terms

  • Andersen Syndrome / diagnosis
  • Andersen Syndrome / therapy*
  • Child
  • Defibrillators, Implantable / adverse effects*
  • Electric Countershock / instrumentation*
  • Electrocardiography, Ambulatory
  • Equipment Failure
  • Female
  • Foreign-Body Migration / diagnostic imaging
  • Foreign-Body Migration / etiology*
  • Humans
  • Radiography
  • Treatment Outcome