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Catecholaminergic Polymorphic Ventricular Tachycardia
From WikiSM
Other Names
- CPVT
Background
- Known cause of polymorphic ventricular tachycardia in younger kids
- CPVT is responsible for 15% of autopsy negative episodes of sudden cardiac death in a young patient [1]
Pathophysiology
- Researchers have identified that changes in intracellular calcium release from the sarcoplasmic reticulum is responsible for CPVT [2]
- They have identified the RyR2 gain of function mutation in 60% of CPVT cases
Clinical Features
- Syncope
- Cardiac arrest
Differential Diagnosis
Risk Factors
- Genetic inheritance
- RYR2 gene is the known gene
Evaluation
- ECG
- EKG in CPVT is typically normal
- Exercise stress test
- Genetic testing
- Indications for genetic testing are when a cardiologist has a clinical suspicion for CPVT based on family history, patient’s clinical history, and expressible phenotype during exercise EKG. Having exercise induced syncope or cardiac arrest in setting of a QTc <460ms should also be considered for CPVT testing
Classification
Management
- Beta blockers
- Flecainide
- ICD if indicated
Complications
- Can potentially lead to sudden death
See Also
References
Created by:
John Kiel on 13 June 2019 09:13:24
Authors:
Last edited:
27 August 2019 11:41:28
Category:
