Jeong Dong Seop, Kim Kyung-Hwan, Kim Jun Sung, Ahn Hyun
Department of Thoracic and Cardiovascular Surgery, Seoul National University Borame Medical Center, Seoul, South Korea.
Ann Thorac Surg. 2009 Jun;87(6):1775-82. doi: 10.1016/j.athoracsur.2009.03.008.
Cardiac involvement in Behçet's disease is a rare but severe complication and presents challenges to cardiac surgeons as a result of late valve detachment or pseudoaneurysms of the aortic root after valve surgery. Few reports have been published on this topic. In this article, clinical data and surgical outcomes in patients with aortic regurgitation attributable to Behçet's disease were analyzed.
Nineteen patients with aortic regurgitation attributable to Behçet's disease were surgically treated between March 1986 and June 2008. There were 15 men and 4 women with ages ranging from 24 to 55 years (mean, 39 +/- 7 years). Mean follow-up duration from index operations was 77.4 +/- 68.1 months (range, 9 to 271 months).
Overall mortality was 47.3% (9 of 19 patients), but no early deaths occurred at index operations. All deaths occurred after second operations, and the causes of death were low cardiac output (n = 6) and sudden aggravation of aortic regurgitation (n = 3). Erythrocyte sedimentation rates and C-reactive protein concentrations were negatively correlated with event-free period. Event-free survival at 13 years was 39.2% +/- 14.1% in patients who underwent aortic root replacement, but this was 4% +/- 3.9% in patients who underwent valve replacement (p = 0.001). Event-free survival at 13 years in patients who were administered immunosuppressive therapies was 33.7% +/- 11.0% and 0% in patients not administered immunosuppressive therapy (p = 0.001).
The mortality in this condition was very high and was found to depend on levels of postoperative inflammatory markers. Aortic root replacement and postoperative immunosuppressive therapy may be helpful.
白塞病累及心脏是一种罕见但严重的并发症，由于瓣膜手术后瓣膜晚期脱离或主动脉根部假性动脉瘤，给心脏外科医生带来了挑战。关于这一主题的报道很少。本文分析了白塞病所致主动脉反流患者的临床资料和手术结果。
1986年3月至2008年6月期间，对19例白塞病所致主动脉反流患者进行了手术治疗。其中男性15例，女性4例，年龄24至55岁（平均39±7岁）。从初次手术开始的平均随访时间为77.4±68.1个月（范围9至271个月）。
总死亡率为47.3%（19例患者中的9例），但初次手术时无早期死亡发生。所有死亡均发生在二次手术后，死亡原因是心输出量低（n = 6）和主动脉反流突然加重（n = 3）。红细胞沉降率和C反应蛋白浓度与无事件生存期呈负相关。接受主动脉根部置换的患者13年无事件生存率为39.2%±14.1%，而接受瓣膜置换的患者为4%±3.9%（p = 0.001）。接受免疫抑制治疗的患者13年无事件生存率为33.7%±11.0%，未接受免疫抑制治疗的患者为0%（p = 0.001）。
这种情况下的死亡率非常高，且发现取决于术后炎症标志物水平。主动脉根部置换和术后免疫抑制治疗可能会有帮助。
