Which clinical history findings are characteristic of Andersen-Tawil syndrome?

Updated: Apr 30, 2018
  • Author: Naganand Sripathi, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Andersen-Tawil syndrome is characterized by variable expression of the triad of dysmorphic features, periodic paralysis, and cardiac arrhythmias. Patients may have short stature, hypertelorism, low-set ears, micrognathia, fifth finger clinodactyly, and scoliosis. Episodic weakness lasting a few hours to several days may arise spontaneously but usually follows physical activity. The periodic paralysis is not associated with myotonia.

Prolonged QT interval and ventricular arrhythmias are the most common cardiac manifestations. Other ECG abnormalities include PVCs, ventricular bigeminy, supraventricular and ventricular tachycardias, prominent U waves, and torsades de pointes. Bidirectional ventricular tachycardia, which is characterized by beat-to-beat alternating QRS axis polarity, is unique to a subset of patients. Patients may be completely asymptomatic. Patients may experience palpitations, syncopal episodes, and cardiac arrest. Sudden cardiac death is less frequent in ATS when compared with the other long QT syndromes.

Andersen-Tawil syndrome should always be considered in any patient with periodic paralysis as facial dysmorphism may be subtle and cardiac symptoms are not always present in spite of an abnormal ECG.


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