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

Abstract 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.