Cardiac Magnetic Resonance Imaging Macroscopic Fibro-Fatty Infiltration of the Myocardium in Pediatric Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. (1st November 2020)

• Record Type: Journal Article
• Authors: Slesnick, Timothy; Parks, W. James; Poulik, Janet; Al-Haddad, Eman; Vickery, Jasmine; Eskarous, Hany; Youssef, Lara; Mangal, Ruchi; Shehata, Bahig M.
• Is Part Of: Fetal and pediatric pathology. Volume 39:Number 6(2020)
• Page Start: 455
• Page End: 466
• Publication Date: 2020-11-01
• Subjects: cardiac magnetic resonance imaging -- CMRI -- fibrofatty infiltration -- ARVC/D -- arrhythmogenic right ventricular cardiomyopathy/dysplasia; Pathology, Molecular -- Periodicals; Pediatrics -- Periodicals; Molecular biology -- Periodicals; Pediatric pathology -- Periodicals; Fetal Diseases -- pathology -- Periodicals; Infant, Newborn, Diseases -- pathology -- Periodicals; Pediatrics -- Periodicals; 618.92007
• DOI: 10.1080/15513815.2019.1675108
• Languages: English
• ISSNs: 1551-3815

Abstract

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.