Ghori M A, Al Sousi Awatif, Al Mahmeed Wael, Ellahham Samer, Ayman Moataz, Augustin Norbert
Department of Cardiac Sciences, Adult Cardiology Department, Sheikh Khalifa Medical City, Abu Dhabi.
J Saudi Heart Assoc. 2013 Apr;25(2):85-9. doi: 10.1016/j.jsha.2013.02.001. Epub 2013 Feb 18.
Behçet's disease (BD) is a multi-system, chronic and relapsing disorder classified as "vasculitic syndrome". It typically affects young adult females between 20 and 40 years of age. There are some typical clinical manifestations associated with this disease, however, at times; rare sign and symptoms pose a challenge to the treating physician and making a definitive diagnosis. Presentations with cardiac symptoms are one of the extremely rare manifestations of the Behçet's disease.
The authors present clinical, laboratory and imaging findings of a patient who presented with a cardiac mass which was the first presenting feature or manifestation of Behçet's disease.
A 19-year-old boy was admitted to our hospital for the investigation of "fever of unknown origin", weight loss, shortness of breath and a scrotal ulcer of recent on-set. X-ray chest and electrocardiograms were inconclusive. Transthoracic echocardiography revealed a right ventricular (RV) mass attached to the interventricular septum measuring 1.5 × 1.5 cms (Panel A). Cardiac MRI identified it as a RV Myxoma. In addition, on CT scan of the chest pulmonary embolism was noted. The patient underwent excision biopsy of the tumor under cardiopulmonary bypass via right atriotomy (Panel B). Histopathology of the mass described it as "an organizing thrombus with a few groups of interrupted myocardial fibers and some infiltration of lymphocytes and plasma cells". Moreover his HLA typing was found positive for HLA-B51 (5). In view of the above findings and associated lesions, the patient was diagnosed as a case of Behçet's disease. The medical management included immunosuppressant and anticoagulation.
Behçet's disease, even in the absence of the typical clinical features, should be considered in the differential diagnosis of right ventricular mass, especially when dealing with young adults from the Mediterranean basin and the Middle-East.
白塞病（BD）是一种多系统、慢性复发性疾病，归类为“血管炎综合征”。它通常影响20至40岁的年轻成年女性。这种疾病有一些典型的临床表现，但有时罕见的体征和症状给治疗医生带来挑战，难以做出明确诊断。出现心脏症状是白塞病极为罕见的表现之一。
作者介绍了一名患者的临床、实验室和影像学检查结果，该患者以心脏肿块为首发特征或表现，而这是白塞病的表现。
一名19岁男孩因“不明原因发热”、体重减轻、呼吸急促和近期出现的阴囊溃疡入院。胸部X线和心电图检查结果不明确。经胸超声心动图显示一个附着于室间隔的右心室（RV）肿块，大小为1.5×1.5厘米（图A）。心脏磁共振成像将其确定为右心室黏液瘤。此外，胸部CT扫描发现有肺栓塞。患者在体外循环下经右心房切开术进行了肿瘤切除活检（图B）。肿块的组织病理学描述为“一个机化血栓，伴有几组中断的心肌纤维以及一些淋巴细胞和浆细胞浸润”。此外，发现他的人类白细胞抗原（HLA）分型HLA - B51呈阳性（5）。鉴于上述发现及相关病变，该患者被诊断为白塞病。药物治疗包括免疫抑制剂和抗凝治疗。
即使没有典型的临床特征，在鉴别诊断右心室肿块时也应考虑白塞病，特别是在面对来自地中海盆地和中东地区的年轻人时。
