Andersen-Tawil syndrome medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

There is no treatment for Andersen-Tawil Syndrome; the mainstay of therapy is to treat the symptoms and manage the patient. Potassium levels play an important role in the management of the symptoms.

• Serum potassium plays an important role in managing the symptoms of the patients with Andersen-Tawil Syndrome.
• If serum potassium levels are <3.0 mmol/L treat the patient with the following:
  • Preferred regimen (1): Oral potassium 20-30 mEq/L with the intervals of every 15-30 minutes until the patient reaches the normal levels.
  • Specific instructions:
    • Physicians who are treating the patient have to keep in mind that anywhere not more than 200 mEq in a 12-hour period is considered to prevent the toxicity.
  • Preferred regimen (2): If intravenous potassium is considered then a 5% mannitol solution in the place of a saline or glucose solution is recommended
  • Specific instructions:
    • Giving IV potassium with saline or glucose solution leads to worsen the weakness.
• While giving the potassium to a patient it is very important to monitor very closely as to avoid secondary hyperkalemia which might leads to diastolic arrest.
• If the patient’s potassium levels are high and causes episodic paralysis it will resolve within an hour.
• High potassium levels can be managed by treating the patient with the following:
  • Preferred regimen (3): Intravenous calcium gluconate

Cardiac manifestations

• Cardiac manifestations like ventricular arrhythmias occurs in patients with Andersen-Tawil Syndrome treat the patient with the following:^[1][2]

• Flecainide should be considered especially in patients who are prone to more frequent ventricular arrhythmias with reduced left ventricular function

• Flecainide is very potent anti arrhythmic which helps with suppressing bidirectional ventricular tachycardia (BVT)^[3]
• Flecainide also helps in reversing tachycardia-induced cardiomyopathy
  • Preferred regimen (1): Flecainide 50 mg PO BID, may increase by 50 mg but do not exceed 300 mg/day.

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