LQTS: Difference between revisions

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LQTS (view source)

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===Physical examination===
===Physical examination===
[[Image:Torsade_de_Pointes.png|right|thumb]]
[[Image:Torsade_de_Pointes.png|500px|right|thumb]]
Patients can present with symptoms of arrhythmias:
Patients can present with symptoms of arrhythmias:
*Fast or slow heart beat
*Fast or slow heart beat
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|'''Eponyme'''
|'''Eponyme'''
|If condition is homozygous: Jervell and Lange-Nielsen syndrome 1
|If condition is homozygous: Jervell and Lange-Nielsen syndrome 1
|
|
|
|}
|}
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450-459 (in males)
450-459 (in males)


>480 during  
>480 during X-ECG
 
X-ECG
|valign="top"|3
|valign="top"|3


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|0.5
|0.5
|-
|-
|colspan="4"|''Total score =1 Low probability; 1.5-3 Intermediate probability; =3.5 High probability''
|colspan="4"|''Total score ≤1 Low probability; 1.5-3 Intermediate probability; ≥3.5 High probability''
|}
|}
Diagnostic criteria by Schwartz et al. (2011)<cite>Schwartz2001</cite>


==Clinical diagnosis==
==Clinical diagnosis==
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===Medication/Other therapies:===  
===Medication/Other therapies:===  
*Beta-blockers are the cornerstone of therapy in LQTS. Beta-blockers even reduce the risk of sudden death in patients in whom a genetic defect has been found, but no QT prolongation is visible on the ECG and also in LQTS3 patients with bradycardia-associated cardiac events <cite>congenital</cite>
*Beta-blockers are the cornerstone of therapy in LQTS. Beta-blockers even reduce the risk of sudden death in patients in whom a genetic defect has been found, but no QT prolon
*[[ICD]] implantation in combination with beta-blockers in LQTS patients with previous cardiac arrest, cardiac [[syncope]] or [[ventricular tachycardia]] while on beta-blockers. ICDs should have pacing possibilities, because arrhythmic episodes are bradycardia-associated in LQTS type 3 and since post-shock pacing is relevant in all other LQTS types.
*Cardiac sympathetic denervation (LCSD) should be considered in the setting of beta-blocker breakthroughs, intolerance to pharmacotherapy and history of appropriate ICD therapies. Surgically, LCSD involves the resection of the lower two-third of the left stellate ganglion and the left-sided sympathetic chain at the level of T2, T3 and T4.




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<biblio>
<biblio>
#Schwartz2001 pmid=11136691
#Schwartz2001 pmid=11136691
#Alders pmid=20301308
#adenosine pmid=16105845
#priori pmid=12736279
#priori pmid=12736279
#genotype pmid=19926013
#moss pmid=17470695
#Alders pmid=20301308
#probands pmid=22042885
#QTc pmid=22083145
#LongQT pmid=20116193
#Adler pmid=22300664
#Adler pmid=22300664
#congenital pmid=15851169
#Shimizu2009 pmid=19926013
#adenosine pmid=16105845
#Moss pmid=17470695
#Sy pmid=22042885
#Schwartz2011 pmid=22083145
#Viskin pmid=20116193
#Shimizu2004 pmid=15851169
</biblio>
</biblio>
Retrieved from "https://www.textbookofcardiology.org/wiki/Special:MobileDiff/2242...2283"

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