Ver el código de «Catecholaminergic polymorphic ventricular tachycardia»

==Background==
*Rare genetic cause of [[syncope]] or sudden death in young patients
*Caused by defect in genes involved in intracellular calcium regulation 
*Untreated, mortality as high as 35% by age 30<ref>https://pedemmorsels.com/catecholaminergic-polymorphic-vtach/</ref>
*Structurally normal heart
*Often confused with torsades de pointes

==Clinical Features==
*Generally presents between 7-9yo, uncommon to present before 2yo
*Often with family history of known CPVT or sudden unexplained death
*[[Polymorphic ventricular tachycardia]] --> [[syncope]]/presyncope, [[palpitations]], death
*PVT induced by:
**Exercise
**Emotional stress
**Any other cause of increased catecholamines (e.g. drugs, physiologic stressors)

==Differential Diagnosis==
*[[Seizure]]
*[[Toxicologic exposure]] (stimulants or depressant)
*[[CO poisoning ]]
*[[Breath-holding spell]]
*[[Tet spell]]
*[[congenital heart disease|Cardiac disease]]
*[[Pregnancy]] (especially [[ectopic]])
*[[Hypoglycemia (peds)|Hypoglycemia]]

{{Syncope causes}}


==Evaluation==
*Baseline [[ECG]] normal or maybe slightly long QTc
*During (symptomatic) catecholaminergic episodes
**Increased HR leads to progressive ventricular ectopy --> isolated PVCs--> bigeminy--> runs of nonsustained vtach--> sustained [[Vtach]]/[[polymorphic ventricular tachycardia]]
*[[Echocardiography]] will show normal heart

==Management==
===Acute===
*IV [[beta blockers]] first line<ref>https://pedemmorsels.com/catecholaminergic-polymorphic-vtach/</ref>
*Consider [[magnesium]]
*Avoid [[epinephrine]]

===Long-term===
*[[Beta blockers]], often [[nadalol]]
*ICD if fail medications or presents with cardiac arrest
*[[Flecainide]]
*[[Verapamil]]
*Left cardiac sympathetic denervation (!)
*Avoid competitive sports and other stimulants

==Disposition==
*Likely admit

==See Also==


==External Links==


==References==
<references/>

[[Category:Cardiology]] [[Category:Pediatrics]]