Torsade-de-pointes syndrome with short coupling interval

Orpha code: 51084OMIM code: 613600

Definition

A rare variant of Torsade de pointes, a polymorphic ventricular tachycardia, which is characterized by a short coupling interval of the first TdP beat on electrocardiogram in the absence of any structural heart disease. It manifests in early adulthood with syncope, often results in ventricular fibrillation and shows a high risk of sudden cardiac death.

Disease data
Classification

Disease

ORPHA code
51084
OMIM code
613600
ICD10 code
I49.8
ICD11 code
-

No additional description.

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