title: [Altered Z-disks of myofibrils in the cardiomyocytes from patients with Ebstein's anomaly].
abstract: The aim of the study was to examine the peculiarities of the changes in Z-bands of myofibrils in the cardiomyocytes from patients with Ebstein's anomaly.
  
  
  METHODS
  Electron microscopy assay of intraoperative biopsies of the right heart chambers in 41 patients aged from 9 months to 57 years was performed.
  
  
  RESULTS
  Some patients exhibited Z-disk alterations of two types in individual cardiomyocytes, namely: local symmetrical bead-like expansions of Z-disks or longitudinal deposits of Z-material of different lengths along the myofibrils. Z-disk alterations were more common in atrial cardiomyocytes than in the ventricle. The presence of Z-disk alterations in the cardiomyocytes correlated with a number of clinical parameters. In particular, the occurrence of longitudinal deposits of Z-material in atrial cardiomyocytes directly correlated with the manifestation of the Wolff-Parkinson-White syndrome in the patients.
  
  
  CONCLUSIONS
  Above characteristic ultrastructural changes in Z-bands of myofibrils in the cardiomyocytes from patients with Ebstein's anomaly have a certain similarity to Z-band diseases in skeletal muscle at sarcomeric protein gene mutations described in the literature, which suggests the mutations in the genes of proteins included in Z-bands of myofibrils in the cardiomyocytes from patients with Ebstein's anomaly.
authors: A.N. Bakulev Scientific Center of Cardiovascular Surgery, Moscow.
authors: I F
authors: IF
authors: Egorova
authors: A.N. Bakulev Scientific Center of Cardiovascular Surgery, Moscow.
authors: E V
authors: EV
authors: Penyaeva
authors: A.N. Bakulev Scientific Center of Cardiovascular Surgery, Moscow.
authors: L A
authors: LA
authors: Bockeria
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009154:Mutation; D032383:Myocytes, Cardiac / Q000473:pathology* / Q000648:ultrastructure; D009210:Myofibrils / Q000473:pathology* / Q000648:ultrastructure; D012518:Sarcomeres / Q000235:genetics / Q000648:ultrastructure*