Andersen-Tawil syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]
Overview
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.
Medical Therapy
Serum potassium management
- 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:
- 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
- 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.
References
- ↑ 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.
- ↑ 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.
- ↑ 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.