title: [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.
authors.forename: L M
authors.initials: LM
authors.lastname: Makarov
authors.forename: V N
authors.initials: VN
authors.lastname: Komoliatova
authors.forename: V O
authors.initials: VO
authors.lastname: Kolosov
authors.forename: Iu A
authors.initials: IuA
authors.lastname: Solokhin
mesh_terms: D002037:Bundle-Branch Block / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000209:etiology / Q000473:pathology*; D003937:Diagnosis, Differential; D004562:Electrocardiography; D017809:Fatal Outcome; D005260:Female; D006329:Heart Conduction System / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D035781:Siblings