白塞病合并心内占位7例及文献复习

Intracardiac Masses in Seven Patients with Behcet′s Disease: a retrospective Study and Literature Review

目的心内占位在白塞病中极为少见,心内血栓、心内膜-心肌纤维化、炎性包块、囊性变和心内膜弹性纤维组织增生等均仅有个例报道。方法对1990年2011年12月于北京协和医院诊断白塞病的572例住院患者进行回顾性分析,发现7例合并心内占位的病例,对其临床表现、治疗及转归进行了分析。结果 7例患者中6例为男性、1例为女性,发现心内占位时19～40岁(平均年龄29.1±8.2岁,中位年龄32岁),此前BD病程为1个月10年(中位时间4年)。所有患者均有心脏外血栓/栓塞的表现:肺动脉是受累最多的血管,5例患者合并肺动脉栓塞;其他血栓/栓塞的部位包括眼动脉(1/7)、上腔/下腔静脉(2/7)、主动脉(2/7)以及双侧髂动脉、双下肢动脉(1/7)。1例患者合并腹主动脉假性动脉瘤。1例为心包腔内多发占位,余6例均为心腔内占位。7例共10处心腔内占位,3处位于左心室,3处位于右心室,4处位于右心房,大小不同,分别附着于心尖部、室间隔或游离壁。结论行手术切除心内占位后未予糖皮质及免疫抑制剂治疗2例患者中,1例很快再次出现右心房占位,另1例在5个月内未复发。7例均于免疫内科确诊后接受糖皮质激素、免疫抑制剂和抗凝治疗,心内占位可完全消失或明显缩小,随访期间均未见复发。

Objective Cardiac mass is a rare manifestation of Behcet＇s disease （BD）. Intracardiac thrombosis, endomyocardiofi- brosis, endocardial fibroelastosis, inflammatory mass, and cystic change have been reported as different entities of cardiac mass in BD. Methods A retrospective review over 20 - year was conducted among 572 BD in - patients of Peking Union Medical College Hospital, which yielded 7 cases of BD with cardiac mass. Their clinical manifestation, treatment and outcome had been summarized. Results Patients included 6 males and 1 female. The mean age by the time of cardiac mass lesion was 29.1 ＋ 8.2years old （ ranging from 19 to 40 yrs, median age 32 yrs）. Disease duration before discovery of cardiac mass varied from 1 month to 10 years （ median 4 years）. All patients had thrombosis or embolisms of sites other than heart. Pulmonary artery was the mostly involved vessel, and 5 patients （71.4%） had pulmonary embolism. Other sites of embolism/thrombosis included ocular artery （1/7） , superior/inferior vena cava （2/7） , aorta （2/7） ,and bilateral iliac arteries with their lower extremities branches （1/7）. One patient had aorta pseudo -aneurysm. Most cardiac masses were found inside heart chambers （6/7）, except masses in one patient localized in pericardial cavity （1/7）. There were three masses found in left ventricle, three masses found in right ventricle, and four masses found in right atrium. Masses with various sizes attached to apex, atrial or ventricular septum, and free wall separately. Conclusion Among the 2 patients who had surgical removal of cardiac mass but without following immunosuppressive therapy, one recurred with right atrium masses, and the other one had no recurrence of cardiac mass at a short term follow - up of 5 months. All patients were finally treated with corticosteroid, immunosuppressive agents and anticoagulation, and all achieved partial or complete resolve of cardiac masses, and none had recurrence during their follow - up.