catecholaminergic polymorphic ventricular tachycardia

An inherited cardiac conduction disorder of early onset (age 7 to 9), which is characterised by episodic syncope occurring during exercise or acute emotion, which triggers ventricular tachycardia, usually followed by spontaneous recovery or less commonly by ventricular fibrillation and sudden death without CPR.
Diagnosis
Reproducible ventricular arrhythmias during exercise stress testing.
EKG
Alternating 180-degree-QRS axis on a beat-to-beat basis, so-called bidirectional VT, and irregular polymorphic VT without a "stable" QRS vector alternans.
Management
Beta-blockers, even in absence of clinical disease; implantable cardioverter-defibrillator for recurrent cardiac arrest, anticoagulation as needed.
Prevent secondary complications
Avoid exacerbating asthma, cardiac-specific beta-blocker: metoprolol is preferred. Follow-up visits with a cardiologist every 6–12 months to monitor therapy.
Avoid competitive sport and strenuous exercise.
Test blood relatives at risk with resting EKG, Holter monitoring, and exercise stress testing.
Genetic counselling
(1) RYR2-related CPVT is autosomal dominant—i.e., each child has a 50% chance of inheriting the mutation.
(2) CASQ2-related CPVT is autosomal recessive—i.e., each parent of an affected child is a carrier.

单词	catecholaminergic polymorphic ventricular tachycardia
释义	catecholaminergic polymorphic ventricular tachycardia catecholaminergic polymorphic ventricular tachycardia An inherited cardiac conduction disorder of early onset (age 7 to 9), which is characterised by episodic syncope occurring during exercise or acute emotion, which triggers ventricular tachycardia, usually followed by spontaneous recovery or less commonly by ventricular fibrillation and sudden death without CPR. Diagnosis Reproducible ventricular arrhythmias during exercise stress testing. EKG Alternating 180-degree-QRS axis on a beat-to-beat basis, so-called bidirectional VT, and irregular polymorphic VT without a "stable" QRS vector alternans. Management Beta-blockers, even in absence of clinical disease; implantable cardioverter-defibrillator for recurrent cardiac arrest, anticoagulation as needed. Prevent secondary complications Avoid exacerbating asthma, cardiac-specific beta-blocker: metoprolol is preferred. Follow-up visits with a cardiologist every 6–12 months to monitor therapy. Avoid competitive sport and strenuous exercise. Test blood relatives at risk with resting EKG, Holter monitoring, and exercise stress testing. Genetic counselling (1) RYR2-related CPVT is autosomal dominant—i.e., each child has a 50% chance of inheriting the mutation. (2) CASQ2-related CPVT is autosomal recessive—i.e., each parent of an affected child is a carrier. AcronymsSeeCPVT
随便看	eat out of house and home eat out of my hand eat out of (one's) hand eat out of one's hand eat out of our hand eat out of (someone's) hand eat out of someone's hand eat out of someone's hand, to eat out of their hand eat out of your hand eat own dog food eatp eatpm eat, pray, love eat pussy eatq-r eatr eatrc eats eat salt eat salt with eat salt with her eat salt with him eat salt with me eat salt with one