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[1]
  • Structurally normal heart

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

Syncope Causes


Evaluation

  • Baseline ECG normal or maybe slightly long QTc
  • During (symptomatic) catecholaminergic episodes
  • Echocardiography will show normal heart

Management

Acute

Long-term

Disposition

  • Likely admit

See Also

External Links

References

Authors: