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一例累及双心室的致心律失常性右室心肌病。

A case of arrhythmogenic right ventricular cardiomyopathy with biventricular involvement.

作者信息

Brandimarte Filippo, Battagliese Alessandro, Pirillo Silvana Petronilla, Mallus Maria Teresa, Manfredi Rosa Maria, Carreras Giovanni

机构信息

San Giovanni Addolorata Community Hospital, Cardiology and Rehabilitation Unit, Department of Medicine, Rome.

出版信息

Monaldi Arch Chest Dis. 2019 Mar 27;89(1). doi: 10.4081/monaldi.2019.1009.

Abstract

We reported a case of a young adult male aged 18 years admitted in our institution for syncope during a basketball match. No previous symptoms were reported. Electrocardiogram (ECG) showed T-wave inversion in the anterior leads and an incomplete right bundle branch block. Surprisingly, a complete echocardiographic evaluation demonstrated the presence of severe right ventricular enlargement with significant wall motion abnormalities, apical aneurysm and reduced systolic function. Cardiac Magnetic Resonance was pathognomonic for a fibro-fatty replacement of both ventricles. We decided for a subcutaneous defibrillator implantation and, after inducing a ventricular fibrillation to test the device status, epsilon wave appeared on the ECG. This clinical scenario depicted an advanced arrhythmogenic right ventricular cardiomyopathy at its first clinical manifestation.

摘要

我们报告了一例18岁的年轻成年男性病例,该患者因在篮球比赛中晕厥而入住我院。既往无相关症状报告。心电图(ECG)显示前壁导联T波倒置及不完全性右束支传导阻滞。令人惊讶的是,完整的超声心动图评估显示存在严重的右心室扩大,伴有明显的室壁运动异常、心尖部室壁瘤及收缩功能降低。心脏磁共振成像显示两心室均有纤维脂肪替代,具有特征性。我们决定植入皮下除颤器,在诱发室颤以测试设备状态后,心电图上出现了epsilon波。这一临床情况描述了致心律失常性右心室心肌病首次临床表现时的晚期状态。

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