Dilated cariomyopathy in infancy. Ultrastructural image analysis for diagnostic purpose

C. Bosman, R. Boldrini, S. Fusilli

Research output: Contribution to journalArticlepeer-review


30 patients in paediatric age have been submitted to endocardial biopsy of the right ventricle, most of them affected by a dilated cardiomyopathy. Our aim was to improve knowledge on this disease using ultrastructural morphometric analysis. An enlargement of myocardial cells with granular aspects of the cytoplasm, known as cloudy swelling, was observed at histological level. Electronmicroscopy showed two main abnormalities: 1) a constant increase of the chondrioma resulting in nuclear deformation and myofibrillary dislocation towards the sarcolemma. 2) disarrangement of thin (actin) and thick (myosin) myofilaments. Such myofibrillary dislocation, designated as fibrillar segregation, suggests how mycardial fibres may loose their ability to contract. The stereological study of these two ultrastructural abnormalities showed that mitochondria are increased in number and size and that the loss of contractile properties of myocardial cells can be related to myofibrillar segregation.

Original languageEnglish
Pages (from-to)807-817
Number of pages11
JournalPathology Research and Practice
Issue number5
Publication statusPublished - 1989

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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