Purkinje fiber dysplasia (histiocytoid cardiomyopathy) with ventricular noncompaction in a savannah kitten.

TitlePurkinje fiber dysplasia (histiocytoid cardiomyopathy) with ventricular noncompaction in a savannah kitten.
Publication TypeJournal Article
Year of Publication2009
AuthorsGelberg HB
JournalVeterinary pathology
Volume46
Issue4
Pagination693-7
Date Published2009 Jul
ISSN1544-2217
KeywordsAnimals, Cardiomyopathies, Cat Diseases, Cats, Fatal Outcome, Female, Histiocytes, Immunohistochemistry, Purkinje Fibers
Abstract

In a 2-month-old female savannah kitten that died unexpectedly, the pathologic findings of significance were restricted to the heart and included abnormal Purkinje fibers and biventricular myocardial trabeculation or noncompaction. The Purkinje fibers were large, angular, and tightly packed. They contained few disorganized myofibrils among a rarified cytoplasm. The fibers were distinct from adjacent myocytes and were immunohistochemically positive for desmin, muscle actin, myoglobin, sarcomeric actin, and chromogranin A. These findings are identical to those that occur in children with histiocytoid cardiomyopathy, a fatal genetic mitochondrial disorder of Purkinje fibers. Ventricular noncompaction likely has a multifactoral cause that results from fetal arrest of ventricular organizational development that might occur in conjunction with, or independent of, histiocytoid cardiomyopathy.

DOI10.1111/j.1365-3164.2011.00980.x
Alternate JournalVet. Pathol.