Duarte Martins1, Caroline Ovaert2, Diala Khraiche3, Nathalie Boddaert4, Damien Bonnet5, Francesca Raimondi6. 1. Hôpital Universitaire Necker Enfants Malades, Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes, M3C, 149, rue de Sèvres, 75743 Paris Cedex 15, France; Hospital de Santa Cruz, Centro Hospitalar Lisboa Ocidental, Av Prof. Reinaldo dos Santos, 2790-134, Carnaxide, Lisboa, Portugal. Electronic address: duartesaraivamartins@gmail.com. 2. Cardiologie pédiatrique et congénitale, AP-HM, Timone enfants, Hopital de la Timone 264 Rue Saint Pierre, 13005, Marseille 05, Provence-Alpes-Côte d'Azur, France. 3. Hôpital Universitaire Necker Enfants Malades, Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes, M3C, 149, rue de Sèvres, 75743 Paris Cedex 15, France. 4. Hôpital Universitaire Necker Enfants Malades, Service de Radiologie pédiatrique, 149, rue de Sèvres, 75743 Paris Cedex 15, France. 5. Hôpital Universitaire Necker Enfants Malades, Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes, M3C, 149, rue de Sèvres, 75743 Paris Cedex 15, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. 6. Hôpital Universitaire Necker Enfants Malades, Unité Médico-Chirurgicale de Cardiologie Congénitale et Pédiatrique, Centre de référence Malformations Cardiaques Congénitales Complexes, M3C, 149, rue de Sèvres, 75743 Paris Cedex 15, France; Hôpital Universitaire Necker Enfants Malades, Service de Radiologie pédiatrique, 149, rue de Sèvres, 75743 Paris Cedex 15, France.
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.
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.
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