Andersen-Tawil syndrome medical therapy: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Andersen-Tawil syndrome}} | {{Andersen-Tawil syndrome}} | ||
{{CMG}}; {{AE}} {{ | {{CMG}}; {{AE}} {{VKG}} | ||
== Overview == | |||
There is no treatment for [[Andersen-Tawil syndrome|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== | ==Medical Therapy== | ||
==== Serum potassium management ==== | |||
* If | |||
* | *[[Serum]] [[potassium]] plays an important role in managing the [[symptoms]] of the patients with [[Andersen-Tawil syndrome|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 [[Intravenous therapy|IV]] [[potassium]] with [[saline]] or [[glucose]] solution leads to worsen the [[Muscle weakness|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|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 therapy|Intravenous]] [[calcium gluconate]] | |||
'''Cardiac manifestations''' | |||
*[[Cardiac]] manifestations like [[ventricular arrhythmias]] occurs in patients with [[Andersen-Tawil syndrome|Andersen-Tawil Syndrome]] treat the patient with the following:<ref name="BökenkampWilde2007">{{cite journal|last1=Bökenkamp|first1=Regina|last2=Wilde|first2=Arthur A.|last3=Schalij|first3=Martin J.|last4=Blom|first4=Nico A.|title=Flecainide for recurrent malignant ventricular arrhythmias in two siblings with Andersen-Tawil syndrome|journal=Heart Rhythm|volume=4|issue=4|year=2007|pages=508–511|issn=15475271|doi=10.1016/j.hrthm.2006.12.031}}</ref><ref name="pmid18621769">{{cite journal| author=Fox DJ, Klein GJ, Hahn A, Skanes AC, Gula LJ, Yee RK | display-authors=etal| title=Reduction of complex ventricular ectopy and improvement in exercise capacity with flecainide therapy in Andersen-Tawil syndrome. | journal=Europace | year= 2008 | volume= 10 | issue= 8 | pages= 1006-8 | pmid=18621769 | doi=10.1093/europace/eun180 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18621769 }}</ref> | |||
==== 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)<ref name="pmid17655675">{{cite journal| author=Pellizzón OA, Kalaizich L, Ptácek LJ, Tristani-Firouzi M, Gonzalez MD| title=Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome. | journal=J Cardiovasc Electrophysiol | year= 2008 | volume= 19 | issue= 1 | pages= 95-7 | pmid=17655675 | doi=10.1111/j.1540-8167.2007.00910.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17655675 }}</ref> | |||
*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== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
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[[Category:Disease]] | {{WikiDoc Sources}} | ||
[[Category:Rare Disease]] | |||
[[Category:Genetic disorders]] | [[Category:Genetic disorders]] | ||
[[Category:Cardiology]] | [[Category:Cardiology]] | ||
Latest revision as of 14:48, 17 February 2020
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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.