What is the diagnostic ECG finding in CPVT?

Bidirectional VT (alternating QRS axis 180 degrees beat-to-beat) during exercise or catecholamine infusion is pathognomonic for CPVT. Polymorphic VT and PVCs appearing at HR >100-130 bpm are also characteristic.

How is CPVT threshold used for therapy assessment?

The arrhythmia threshold HR during baseline testing is compared to threshold HR after starting beta-blockers or flecainide. Effective therapy raises the threshold. Persistence at low HR indicates inadequate control.

What genes cause CPVT?

RYR2 (ryanodine receptor 2) accounts for ~65% of CPVT. CASQ2 (calsequestrin 2) causes recessive CPVT. CALM1/2/3 calmodulin variants cause severe CPVT phenotype with early presentation.

CPVT Exercise Inducibility Threshold

Determine catecholaminergic polymorphic VT (CPVT) diagnosis and arrhythmia threshold heart rate during exercise stress testing. Bidirectional VT at <130 bpm is highly diagnostic.

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