[Altered Z-disks of myofibrils in the cardiomyocytes from patients with Ebstein's anomaly].

Abstract

UNLABELLED:

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.

MATERIAL AND 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: I F Egorova, E V Penyaeva, L A Bockeria
• Journal: Arkhiv patologii (Arkh Patol) 2015 Nov-Dec Vol. 77 Issue 6 Pg. 3-8 ISSN: 0004-1955 [Print] Russia (Federation)
• PMID: 26841643 (Publication Type: English Abstract, Journal Article)

Topics

• Adolescent
• Adult
• Child
• Child, Preschool
• Ebstein Anomaly (genetics, physiopathology)
• Female
• Heart Ventricles (pathology)
• Humans
• Infant
• Male
• Middle Aged
• Mutation
• Myocytes, Cardiac (pathology, ultrastructure)
• Myofibrils (pathology, ultrastructure)
• Sarcomeres (genetics, ultrastructure)