Cardiac Magnetic Resonance Imaging Macroscopic Fibro-Fatty Infiltration of the Myocardium in Pediatric Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.
Fetal Pediatr Pathol
; 39(6): 455-466, 2020 Dec.
Article
in En
| MEDLINE
| ID: mdl-31625461
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Arrhythmogenic Right Ventricular Dysplasia
Type of study:
Prognostic_studies
Limits:
Child
/
Humans
Language:
En
Journal:
Fetal Pediatr Pathol
Journal subject:
PATOLOGIA
/
PEDIATRIA
Year:
2020
Document type:
Article
Affiliation country:
United States