A case of a rare disease - Andersen-Tawil the syndrome (ATS) is presented. Diagnosis of ATS, 7-th molecular-genetic variant of long QT syndrome was made basing on the characteristic clinical picture (periodic stress induced syncopal states), data of ECG and its Holter monitoring (pronounced QT prolongation, bouts of polymorphic bidirectional ventricular tachycardia), typical dysmorphic features (low-set ears, small mandible, brachydactyly, fifth-digit clinodactyly). However mutation of the KCNJ2 gene typical for this variant was not detected. Problems of pathogenesis, diagnostics, and treatment of the disease are discussed with special stress on class IC antiarrhythmic drugs.
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