Andersen-Tawil syndrome secondary prevention

Prophylactic treatment aimed at reduction of attack frequency and severity can be achieved, as in other forms of periodic paralysis, with the following:

• Lifestyle and dietary modifications to avoid known triggers
• Use of carbonic anhydrase inhibitors (acetazolamide 250-500 mg/1-2x/day or dichlorphenamide 50-100 mg/1-2x/day)
• Daily use of slow-release potassium supplements, which may also be helpful in controlling attack rates in individuals prone to hypokalemia. Elevating the serum potassium concentration (>4 mEq/L) has the added benefit of narrowing the QT interval, thus reducing the risk of LQT-associated arrhythmias.
• An implantable cardioverter-defibrillator in individuals with tachycardia-induced syncope
• Empiric treatment with flecainide should be considered for significant, frequent ventricular arrhythmias in the setting of reduced left ventricular function.

• Affected individuals should avoid medications known to prolong QT intervals.
• Salbutamol inhalers, which may be used in the treatment of primary hyperkalemic periodic paralysis, should be avoided because of the potential for exacerbation of cardiac arrhythmias.
• Thiazide and other potassium-wasting diuretics may provoke drug-induced hypokalemia and could aggravate the QT interval.

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of Andersen-Tawil syndrome (ATS) include avoidance of some antiarrhythmic drugs and anesthetic precautions.

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of Andersen-Tawil syndrome (ATS) include:

• Antiarrhythmic drugs: Antiarrhythmic drugs should be used with extra caution as the drugs may deteriorate the neuromuscular symptoms. Which include
  • Lidocaine
  • Mexiletine
  • Propafenone
  • Quinidine
• Anesthetic care: Care should be taken to avoid malignant hyperthermia when giving anesthesia to the patients of Andersen-Tawil syndrome (ATS).

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