catecholaminergic polymorphic ventricular tachycardia

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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.

Reproducible ventricular arrhythmias during exercise stress testing.

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.

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.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.
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The mean age at the onset of CPVT symptoms for the 12 cases was 8.4 [+ or -] 3.2 years (4.0-13.7 years).
Death is the first symptom of the disease in 30% of cases with CPVT (24).
This agreed with clinical data, according to which CPVT type II patients had bradycardia at rest.
Following WES and variant annotation, variant filtration involving the exclusion of all noncoding regions and synonymous variants (ie, a DNA nucleotide alteration that does not alter the amino acid sequence of the protein) and gene-specific surveillance of all known LQTS, CPVT, BrS, HCM, DCM, and ACM genes (90 total; Table 1 and Figure 1) were performed to identify possible pathogenic mutation(s).
Majority of CPVT cases arise from mutations in the gene coding for cardiac ryanodine receptor, RYR2.
In CPVT, exercise or stress results in stimulation of beta adrenergic receptors, promoting cyclic AMP mediated phosphorylation of ryanodine receptors, leading to further intracellular calcium overload.
The acquired business will operate as CPvT Demansys Energy Pte Ltd, led by its CEO Vijay Sirse, who is also CEO and chairman of the vendor, the buyer said.
London, Mar 30 (ANI): Vanderbilt University Medical Center researchers have found that a drug can prevent potentially lethal arrhythmias caused due to exercise or stress, called CPVT.
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