Myocardial inflammation detected by cardiac MRI in Arrhythmogenic right ventricular cardiomyopathy: A paediatric case series. (15th November 2018)

• Record Type: Journal Article
• Authors: Martins, Duarte; Ovaert, Caroline; Khraiche, Diala; Boddaert, Nathalie; Bonnet, Damien; Raimondi, Francesca
• Is Part Of: International journal of cardiology. Volume 271(2018)
• Page Start: 81
• Page End: 86
• Publication Date: 2018-11-15
• Subjects: Cardiology -- Periodicals; Electronic journals; 616.12
• DOI: 10.1016/j.ijcard.2018.05.116
• Languages: English
• ISSNs: 0167-5273

Abstract

Abstract: Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited myocardial disease with an elusive association with myocardial inflammation. A myocarditis phenotype has been well established without systematic evidence of a viral trigger. We intend to study the relationship between myocardial inflammation detected by Cardiac magnetic resonance (CMR) and ARVC in a paediatric population. Methods: Retrospective case series of all patients <18 years admitted to two CMR units for clinical suspicion of myocarditis from March 2012 to June 2017 who had genetic testing for inherited cardiomyopathies including analysis for known ARVC genes. Results: Six patients were identified experiencing myocarditis-like episodes with chest pain and troponin elevation. All had CMR evidence of active myocardial inflammation often affecting the left ventricle without identification of an infectious trigger. These episodes were likely exercise-induced in 50% of our patients and were multiple in all but one. Conclusion: We provide evidence that ARVC can present as recurrent myocarditis-like episodes with CMR evidence of myocardial inflammation despite absent infectious trigger in children. We believe they represent an active hot phase of the disease and may lead to disease progression. Highlights: Paediatric Patients presenting with clinical and mri evidence of myocarditis may later develop diagnostic criteria for Arrhythmogenic Right Ventricular Cardiomyopathy. These myocarditis episodes seem to be exercise-induced rather than infectious, are often multiple and may lead to disease progression. We believe these episodes to be active phases of the disease and part of its natural history.