[Andersen-Tawil syndrome. Efficacy of class IC drugs].

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.

[Sudden death of two sisters in a family with combined progressive impairment of cardiac conduction system].

Sudden cardiac death (SCD) of two sisters from a family with combined progressive impairment of cardiac conduction system (CCS) is described. These two sisters (hetero-ovular twins) died suddenly in a kindergarten at the background of physical and emotional effort in the age of 4.8 and 5 years. Resuscitation and electrical defibrillation conducted in one of them after 20 min of heart arrest were not effective. Autopsy gave no clear-cut data relative to pathology of the heart and other organs. Analysis of preserved ECGs showed that in the age of 8 months both sisters had sharp leftward/upward deviation of electric axis of the heart (-41 in one sister and -43 in another). In 2 years these deviations became even more pronounced reaching -63. This was combined with small q-waves and tall R waves in leads I and aVL what corresponded to signs of block of the anterior branch of bundle of His and could be a manifestation of hereditary disease with progressive CCS involvement first of all of the Lev-Lenegre disease.