Supraventricular Tachy-arrhythmia Revealing an Arrhythmogenic Right Ventricle Cardiomyopathy in An Adolescent: Case Report

B. Mohamed Ghoulame, H. Siyam, P. M. Mulendele, S.M. Obeidat, M. Bouziane, M. Haboub, S. Arous, G. Bennouna, A. Drighil, R. Habbal
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Abstract

Arrhythmogenic right ventricular cardiomyopathy is a genetic disease that affects young people. As its name indicates, it carries a risk of ventricular rhythm disturbances and sudden death, particularly during sporting efforts emphasizing the need for early diagnosis of the disease. The most frequently observed symptoms are palpitations, syncope, clinical signs of right heart failure and cardiac arrest. These clinical manifestations, as well as the progression and prognosis of the disease have been well studied and described in the literature in adult population unlike in children where it has not been well elucidated. Its diagnosis is based on the morphological characteristics of the right ventricle during cardiac ultrasound and especially on cardiac MRI. The extent of morphological abnormalities and the frequency of occurrence of rhythm disorders make it possible to stratify the disease risk. High-risk patients should benefit from implantable cardioverter defibrillator implantation. Treatment is based on moderate physical exercise, beta blockers medical therapy and an implantable cardioverter defibrillator in certain cases. We report a case of a 15-year-old child, with a history of first-degree consanguinity and undocumented heart disease in the father who consulted for chest pain over 8 days. The clinical presentation, electrical signs, cardiac ultrasound and imaging made it possible to make the diagnosis of ARVC.
揭示青少年心律失常性右室心肌病的室上性心动过速:病例报告
致心律失常性右室心肌病是一种影响年轻人的遗传病。正如它的名字所示,这种疾病具有心室节律紊乱和猝死的风险,尤其是在体育运动中,这就强调了早期诊断这种疾病的必要性。最常见的症状是心悸、晕厥、右心衰竭的临床表现和心脏骤停。这些临床表现以及疾病的进展和预后在成人文献中都有很好的研究和描述,但在儿童文献中却没有很好的阐释。其诊断依据是心脏超声,尤其是心脏磁共振成像中右心室的形态特征。根据形态异常的程度和心律失常发生的频率可以对疾病风险进行分层。高危患者应从植入式心脏除颤器中获益。治疗的基础是适度的体育锻炼、β受体阻滞剂的药物治疗以及在某些情况下植入心律转复除颤器。我们报告了一例 15 岁儿童的病例,其父亲有一级血缘关系,且无心脏病史,因胸痛就诊 8 天。根据临床表现、心电体征、心脏超声和影像学检查,可以确诊为 ARVC。
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