The mystery behind recurrent pericardial effusions: A hidden case of arrhythmogenic right ventricular cardiomyopathy

Q4 Medicine

Radiology Case Reports Pub Date : 2025-04-26 DOI:10.1016/j.radcr.2025.03.085

Salma Bouyaddid , Yasmine Ouaddouh , Nabila Ismaili , Noha El ouafi , Zakaria Bazid

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Abstract

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.

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反复心包积液背后的奥秘：一例隐藏的致心律失常性右室心肌病

心律失常性右室心肌病（ARVC）是一种罕见但潜在危及生命的遗传性疾病，其特征为纤维脂肪性心肌替代、心室功能障碍和心律失常。由于其表型异质性，提出了一个重大的诊断挑战。虽然ARVC的主要特征是电不稳定和心源性猝死风险升高，但心包积液是ARVC的一种罕见表现，可能由心肌重构和局部炎症过程之间的复杂相互作用引起，可能掩盖潜在的心脏病理，导致对该疾病的认识延迟。我们报告一例复发不明原因的心包积液在一个年轻的女性患者表现为呼吸困难和胸痛。临床检查发现心音不清。尽管最初使用秋水仙碱治疗，但积液进展，需要心包穿刺，产生血清学渗出，感染、细胞学和自身免疫检查均阴性。经胸超声心动图显示右心室扩张伴严重三尖瓣反流。心脏MRI证实右心室运动不全，RVEF为40%，小梁明显。根据2010年工作组标准，明确确定了心律失常性右室心肌病（ARVC）的诊断。本病例强调了考虑ARVC的重要性，即使在没有典型心律失常症状的情况下，仍有不明原因的复发性心包积液和右心室异常。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Radiology Case Reports Medicine-Radiology, Nuclear Medicine and Imaging

CiteScore

1.10

自引率

0.00%

发文量

1074

审稿时长

30 days

期刊介绍： The content of this journal is exclusively case reports that feature diagnostic imaging. Categories in which case reports can be placed include the musculoskeletal system, spine, central nervous system, head and neck, cardiovascular, chest, gastrointestinal, genitourinary, multisystem, pediatric, emergency, women''s imaging, oncologic, normal variants, medical devices, foreign bodies, interventional radiology, nuclear medicine, molecular imaging, ultrasonography, imaging artifacts, forensic, anthropological, and medical-legal. Articles must be well-documented and include a review of the appropriate literature.