Bouyaddid Salma, Ouaddouh Yasmine, Ismaili Nabila, El Ouafi Noha, Bazid Zakaria
Faculty of Medicine and Pharmacy, Mohammed Ist University, Oujda, Morocco.
Department of Cardiology, Mohammed VI University Hospital/Mohammed I University Oujda Morocco.
Radiol Case Rep. 2025 Apr 26;20(7):3414-3419. doi: 10.1016/j.radcr.2025.03.085. eCollection 2025 Jul.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare but potentially life- threatening genetic disorder characterized by fibrofatty myocardial replacement, ventricular dysfunction, and arrhythmias. Presenting a significant diagnostic challenge due to its phenotypic heterogeneity. While the cardinal features of ARVC are electrical instability and an elevated risk of sudden cardiac death, pericardial effusion, an infrequent manifestation of ARVC, potentially arising from complex interactions between myocardial remodeling and local inflammatory processes, can obscure the underlying cardiac pathology, causing a delayed recognition of the disease. We report a case of recurrent unexplained pericardial effusions in a young female patient presenting with dyspnea and chest pain. Clinical examination revealed muffled heart sounds. Despite initial management with colchicine, the effusion progressed, requiring pericardiocentesis, yielding sero-hematic transudate with negative infectious, cytological, and autoimmune workups. Transthoracic echocardiography revealed a dilated right ventricle with severe tricuspid regurgitation. Cardiac MRI confirmed right ventricular akinesia, an RVEF <40%, and prominent trabeculations. Based on the 2010 Task Force criteria, the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) was definitively established. This case highlights the importance of considering ARVC in patients with unexplained recurrent pericardial effusions and right ventricular abnormalities, even in the absence of typical arrhythmic symptoms.
致心律失常性右室心肌病（ARVC）是一种罕见但可能危及生命的遗传性疾病，其特征为心肌纤维脂肪组织替代、心室功能障碍和心律失常。由于其表型异质性，带来了重大的诊断挑战。虽然ARVC的主要特征是电不稳定和心脏性猝死风险升高，但心包积液作为ARVC的一种罕见表现，可能源于心肌重塑与局部炎症过程之间的复杂相互作用，会掩盖潜在的心脏病理情况，导致疾病的诊断延迟。我们报告一例年轻女性患者反复出现不明原因的心包积液，伴有呼吸困难和胸痛。临床检查发现心音减弱。尽管最初使用秋水仙碱治疗，但积液仍进展，需要进行心包穿刺术，抽出的是血清血性漏出液，感染、细胞学和自身免疫检查均为阴性。经胸超声心动图显示右心室扩张，伴有严重的三尖瓣反流。心脏磁共振成像证实右心室运动减弱，右室射血分数<40%，小梁明显。根据2010年工作组标准，明确诊断为致心律失常性右室心肌病（ARVC）。该病例强调了在不明原因反复出现心包积液和右心室异常的患者中考虑ARVC的重要性，即使没有典型的心律失常症状。
