■心脏并发症发生在1-6%的Behçet病(BD)病例中,心内血栓是最常见的并发症。心内膜纤维化,少见,偶尔与心内血栓有关,在<20例BD报告中报告,其中,根据超声心动图描述了3例模拟Ebstein病。我们介绍了文献中的第一例,一例34岁的BD患者被诊断为多种心血管并发症,强调这种病理的诊断和治疗具有挑战性,尤其是抗凝治疗。
■一个34岁的男人,诊断为BD,因咯血被送到急诊室.胸部诊断的肺动脉动脉瘤伴多发性动脉血栓的计算机断层扫描研究,与右心室和心房多发心内血栓相关。超声心动图证实右心室和心房中存在大量血栓,并显示右心室过度扩张和三尖瓣后小叶高度插入,导致与Ebstein病相容的中度三尖瓣关闭不全。随后心脏MRI显示右心室纤维化与心内膜心肌纤维化和心肌炎后遗症一致,也被描述为BD罕见的心脏表现。在抗凝治疗和免疫抑制药物治疗下,患者的预后良好。
■在患有BD的单个患者中可以发生多种心血管并发症的关联。右心室心内膜纤维化是血栓形成和随后肺栓塞的基质;纤维化延伸到三尖瓣,诱导Ebstein样形态。
UNASSIGNED: Cardiac complications occur in 1-6% of cases of Behçet disease (BD) with intracardiac thrombus being the most frequent complication. Endomyocardial fibrosis, less common and occasionally associated with intracardiac thrombus, is reported in <20 case reports of BD, among which, three cases are described to mimic Ebstein disease based on echocardiography. We present the first case in the literature of a 34-year-old man with BD diagnosed with multiple cardiovascular complications, highlighting the challenging diagnosis and treatment of this pathology, especially regarding anticoagulation therapy.
UNASSIGNED: A 34-year-old man, diagnosed with BD, presented to the Emergency Room with haemoptysis. Computed tomography study of the thorax diagnosed pulmonary arterial aneurysm with multiple arterial thrombi, associated with multiple intracardiac thrombi in the right ventricle and atrium. The echocardiography confirmed the presence of voluminous thrombi in the right ventricle and atrium and showed hypertrabeculation of the right ventricle and a high insertion of the posterior leaflet of the tricuspid valve inducing a moderate tricuspid insufficiency compatible with an Ebstein disease. The cardiac MRI later revealed right ventricular fibrosis consistent with endomyocardial fibrosis and sequelae of myocarditis, also described as BD rare cardiac manifestations. The patient had a favourable outcome under anticoagulant treatment and immunosuppressive drugs.
UNASSIGNED: The association of multiple cardiovascular complications can occur in a single patient with BD. The endomyocardial fibrosis in the right heart chambers acting as a substrate for thrombus formation and subsequent pulmonary embolism; fibrosis extending to the tricuspid valve inducing an Ebstein-like morphology.