以心脏瓣膜病变为突出表现的白塞病10例临床分析

目的 总结以心脏瓣膜病变为突出表现的白塞病患者的临床特点,提高对此类疾病的认识.方法 收集北京协和医院1999年6月至2009年6月以心脏瓣膜病变为突出表现并诊断为白塞病的病例共10例的临床资料,回顾性分析其临床表现、超声心动图资料及治疗情况.结果 白塞病合并主动脉瓣关闭不全共10例,占同期住院白塞病病例的6%.患者平均年龄36.5岁.心脏症状隐匿出现,平均病程为6年.10例患者中5例符合1990年ISG白塞病诊断标准,其中3例在发现主动脉瓣关闭不全前已经被确诊为白塞病;2例虽临床表现符合白塞病诊断标准,但未被医生认识而延误诊断.另5例患者主动脉瓣膜病变突出而白塞的其他临床表现并不全面,经专家会诊、手术病理及排除其他疾病后临床诊断为白塞病.超声心动图主要发现为重度主动脉瓣关闭不全,合并升主动脉扩张或升主动脉瘤,主动脉根部无回声区、主动脉瓣冗长或脱垂、赘生物及穿孔或撕裂等.3例患者行手术治疗共7例次,单纯主动脉瓣置换手术术后均发生机械瓣瓣周漏,2例在激素和免疫抑制剂治疗情况下进行Bentall手术和心脏移植手术,情况良好.结论 白塞病合并心脏瓣膜病变是临床少见处理困难的严重疾病,目前的诊断标准可能不利于此病的早期发现和治疗.超声心动图对诊断有一定帮助,对于不明原因的主动脉瓣关闭不全患者要考虑到白塞病的可能,并注意与感染性心内膜炎鉴别.治疗上改良手术方式,加强免疫抑制治疗,尝试新的治疗手段可能能改善此类患者的预后.

Cardiac valve involvement in Behcet's disease: a clinical study of 10 patients

Objective To assess the clinical features in patients with cardiac valve lesions associated with Behcet's disease (BD). Methods We retrospectively reviewed the clinical data of 10 BD patients with cardiac valve lesions who were admitted to Peking Union Medical College Hospital (PUMCH)during June 1999 to June 2009. Results Aortic regurgitation occured in 6% of patients with BD in PUCMH. Patients included 8 male and 2 female with the mean age of 36.5. All the patients had occult onset cardiac symptoms with an average length of clinical course of 6 years. 5 patients fulfilled the ISG diagnostic criteria for BD and another 5 patients diagnosed by experts. The main echocardiography findings were severe aortic regurgitation, aneurysmal dilatations of ascendan aorta, echo-free space at the aortic root, aortic valve prolapse, meshlike mass incorporating aortic cusp, aortic valve perforation, et al. 3 patients underwent 7operations. 5 simple aortic valve replacement (AVR) surgeries resulted in severe perivalvular leakage. 2patients underwent Bentall and heart transplant surgeries respectively with perioperative immunosuppressive therapy had no complications. Conclusion Cardiac valve involvement in BD is a rare but critical problem that requires a timely diagnosis and management. The current diagnostic criteria may have possibilities of delayed diagnosis of such problem. Echocardiography seems to be helpful for the timely diagnosis. The immunosuppressive therapy and Bentall type operations may be essential for improving the treatment outcome of BD with cardiac valve lesions.