Li X, Wen X, Xu J, Lin Q, Liu L
Department of Cardiovascular Medicine, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Research Institute of Blood Lipid and Atherosclerosis, Central South University, Changsha, Hunan, China.
Neth Heart J. 2022 Mar;30(3):172-180. doi: 10.1007/s12471-021-01567-6. Epub 2021 Apr 20.
Aortic regurgitation is the most common cardiovascular damage in Chinese patients with Behçet's disease (BD) and is usually associated with aortic disease. These patients are easily misdiagnosed, and their prognosis is poor, even after surgical treatment. This study aimed to analyse potential factors that can improve the prognosis of BD patients with aortic regurgitation and/or aortic involvement.
Twenty-two patients with diagnosed or suspected BD as well as aortic regurgitation and/or aortic involvement in our hospital from 2012 through 2017 were collected in this study. Their clinical characteristics were listed, and the diagnosis of BD was evaluated by two different criteria sets. The influences of surgical treatment and immunosuppressive therapy (IST) on their prognosis were also explored.
The diagnostic positive rate of the International Criteria for Behçet's Disease was higher than that of the International Study Group criteria (kappa value 0.31, p < 0.05), indicating that the diagnostic consistency between the criteria sets was poor. There was no significant difference in survival between patients who had undergone ≤ 1 operation and those with ≥ 2 operations. Aortic valve replacement alone or in combination with aortic root replacement had no significant effect on the incidence of reoperation or death, but IST did significantly reduce this incidence (p < 0.05). However, there was no significant difference in the occurrence of reoperation or death between preoperative and postoperative IST versus postoperative IST only.
IST significantly improved the prognosis of BD patients with aortic regurgitation and/or aortic involvement.
主动脉瓣关闭不全是中国白塞病（BD）患者最常见的心血管损害，通常与主动脉疾病相关。这些患者容易被误诊，即使经过手术治疗，其预后也较差。本研究旨在分析可改善BD合并主动脉瓣关闭不全和/或主动脉受累患者预后的潜在因素。
本研究收集了2012年至2017年在我院确诊或疑似患有BD以及合并主动脉瓣关闭不全和/或主动脉受累的22例患者。列出了他们的临床特征，并通过两种不同的标准集对BD诊断进行了评估。还探讨了手术治疗和免疫抑制治疗（IST）对其预后的影响。
白塞病国际标准的诊断阳性率高于国际研究组标准（kappa值0.31，p<0.05），表明两组标准之间的诊断一致性较差。接受≤1次手术的患者与接受≥2次手术的患者生存率无显著差异。单纯主动脉瓣置换或联合主动脉根部置换对再次手术或死亡的发生率无显著影响，但IST确实显著降低了这一发生率（p<0.05）。然而，术前和术后IST与仅术后IST在再次手术或死亡的发生率上无显著差异。
IST显著改善了BD合并主动脉瓣关闭不全和/或主动脉受累患者的预后。
