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

• Published in: Fetal and pediatric pathology Vol. 39; no. 6; pp. 455 - 466
• Main Authors: Slesnick, Timothy, Parks, W. James, Poulik, Janet, Al-Haddad, Eman, Vickery, Jasmine, Eskarous, Hany, Youssef, Lara, Mangal, Ruchi, Shehata, Bahig M.
• Format: Journal Article
• Language: English
• Published: England Taylor & Francis 01.11.2020
• Subjects: arrhythmogenic right ventricular cardiomyopathy/dysplasia; Arrhythmogenic Right Ventricular Dysplasia - diagnostic imaging; ARVC/D; Biopsy; cardiac magnetic resonance imaging; Child; CMRI; fibrofatty infiltration; Heart Ventricles - diagnostic imaging; Humans; Magnetic Resonance Imaging; Myocardium; fibrofatty infiltration; ARVC/D; cardiac magnetic resonance imaging; arrhythmogenic right ventricular cardiomyopathy/dysplasia; CMRI
• ISSN: 1551-3815; 1551-3823
• DOI: 10.1080/15513815.2019.1675108

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.