Andersen-Tawil syndrome medical therapy

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Andersen-Tawil syndrome Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Charmaine Patel, M.D. [2]; Raviteja Guddeti, M.B.B.S. [3]

Overview

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

Medical Therapy

Serum potassium management

Cardiac manifestations

Flecainide

  • 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.
          • Note (1):
    • 2.2 'Other Organ system involved 2'
      Note (1):
      Note (2):
      Note (3):
      • 2.2.1 Adult
        • Parenteral regimen
          • Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
          • Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
          • Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
        • Oral regimen
          • Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
          • Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
          • Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
          • Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
          • Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
          • Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
      • 2.2.2 Pediatric
        • Parenteral regimen
          • Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
          • Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
          • Alternative regimen (2):  drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
        • Oral regimen
          • Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
          • Preferred regimen (2): drug name 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
          • Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
          • Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
          • Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
          • Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)

Medical Therapy

Management of individuals with ATS requires the coordinated input of a neurologist familiar with the treatment of periodic paralysis and a cardiologist familiar with the treatment of cardiac arrhythmias. To date, no randomized clinical therapeutic trials have been conducted on ATS.

Management of attacks of episodic weakness depends on the associated serum potassium concentration:

  • If the serum potassium concentration is low (<3.0 mmol/L), administration of oral potassium (20-30 mEq/L) every 15-30 minutes until the serum concentration normalizes often shortens the attack. Monitoring of serum potassium concentrations and ECG may be useful during potassium replacement therapy in an emergency setting to avoid secondary hyperkalemia.
  • Attacks of weakness when serum potassium concentration is high usually resolve within 60 minutes. Episodes may be shortened by ingesting carbohydrates or continuing mild exercise. Intravenous calcium gluconate is rarely necessary for management in an individual seen in an emergency setting.

Vasovagal syncope in individuals with ATS mandates a careful cardiology assessment.

References

  1. Bökenkamp, Regina; Wilde, Arthur A.; Schalij, Martin J.; Blom, Nico A. (2007). "Flecainide for recurrent malignant ventricular arrhythmias in two siblings with Andersen-Tawil syndrome". Heart Rhythm. 4 (4): 508–511. doi:10.1016/j.hrthm.2006.12.031. ISSN 1547-5271.
  2. Fox DJ, Klein GJ, Hahn A, Skanes AC, Gula LJ, Yee RK; et al. (2008). "Reduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome". Europace. 10 (8): 1006–8. doi:10.1093/europace/eun180. PMID 18621769.
  3. Pellizzón OA, Kalaizich L, Ptácek LJ, Tristani-Firouzi M, Gonzalez MD (2008). "Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome". J Cardiovasc Electrophysiol. 19 (1): 95–7. doi:10.1111/j.1540-8167.2007.00910.x. PMID 17655675.


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