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Cardiac Magnetic Resonance Imaging Macroscopic Fibro-Fatty Infiltration of the Myocardium in Pediatric Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.

Authors :
Slesnick T
Parks WJ
Poulik J
Al-Haddad E
Vickery J
Eskarous H
Youssef L
Mangal R
Shehata BM
Source :
Fetal and pediatric pathology [Fetal Pediatr Pathol] 2020 Dec; Vol. 39 (6), pp. 455-466. Date of Electronic Publication: 2019 Oct 18.
Publication Year :
2020

Abstract

Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited, progressive form of cardiomyopathy, which is characterized by fibrofatty replacement of the myocardium. While the gold standard for diagnosis remains pathologic evaluation of biopsy, advances in noninvasive imaging, including cardiac magnetic resonance imaging (CMRI), have led to improved clinical diagnosis. Case report: We report three additional cases of pediatric patients that have pathologically confirmed ARVC/D with CMRI images, demonstrating extensive macroscopic fatty infiltration of the right and left ventricular myocardium. The identification using CMRI allowed timely transplantation and patient survival. Conclusion: Our study is designed to highlight how fibrofatty changes are minimal using CMRI in the pediatric population and how this can be a valuable tool to provide an additional method of diagnosis.

Details

Language :
English
ISSN :
1551-3823
Volume :
39
Issue :
6
Database :
MEDLINE
Journal :
Fetal and pediatric pathology
Publication Type :
Academic Journal
Accession number :
31625461
Full Text :
https://doi.org/10.1080/15513815.2019.1675108