BACKGROUND Patients with hemophilia A can develop joint hemarthroses, degenerative changes, and eventually undergo total shoulder arthroplasty (TSA). Few data exist concerning complications and prosthesis survival after TSA in this population. QUESTIONS/PURPOSES (1) Is hemophilia A associated with more bleeding and thromboembolic adverse events after TSA relative to matched controls? (2) Is 5-year TSA prosthesis survival reduced in patients with hemophilia A compared with matched controls? METHODS The 2010 to 2022 PearlDiver M161 database was used to identify patients who underwent primary anatomic or reverse TSA. Given that the X-linked recessive condition hemophilia A presents nearly exclusively in males, male patients with hemophilia A who underwent TSA were matched 1:10 with male patients without hemophilia who underwent TSA based on age and Elixhauser comorbidity index (ECI). This yielded 73 patients with hemophilia A who underwent TSA who were matched 1:10 with 729 patients without hemophilia. Ninety-day adverse events were compared with multivariable analysis. Revision within 5 years was assessed using Kaplan-Meier analysis. RESULTS Compared with the control cohort, patients with hemophilia had greater odds of bleeding issues (hematoma, OR 6.8 [95% CI 3.0 to 15.3]; p < 0.001; anemia, OR 2.5 [95% CI 1.5 to 4.2]; p < 0.001, transfusion, OR 5.0 [95% CI 2.4 to 10.3]; p < 0.001), venous thromboembolic events (VTE) (OR 1.9 [95% CI 1.1 to 3.1]; p = 0.01), and prosthetic loosening (OR 3.5 [95% CI 1.4 to 8.0]; p = 0.004). Based on available data, 5-year implant survival was not different in patients with hemophilia (97.3% [95% CI 93.6% to 100.0%]) relative to matched controls (95.2% [95% CI 93.4% to 97.2%]; p = 0.60). CONCLUSION The elevated risks of both 90-day bleeding complications (hematoma, anemia, and transfusion) and VTE (DVT and PE) in patients with hemophilia emphasize the special challenges of carefully balancing factor replacement and VTE prophylaxis pre-, intra-, and postoperatively on an individual patient basis with careful hematologist coordination. Further study on Factor VIII levels and targets as well as tranexamic acid and VTE prophylaxis in this population is necessary to provide further guidance. Furthermore, 5-year implant survival was not different between patients with hemophilia and matched controls (patients without hemophilia) based on available data, suggesting that TSA survivorship remains durable and may be offered to patients in this population as indicated. LEVEL OF EVIDENCE Level III, therapeutic study.

中文翻译：

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A 型血友病患者的全肩关节置换术：术后出血和血栓栓塞事件的几率更大，但 5 年植入物存活率没有差异。


背景甲型血友病患者可能会出现关节积血、退行性改变，并最终接受全肩关节置换术（TSA）。关于该人群 TSA 后并发症和假体存活率的数据很少。问题/目的 (1) 相对于匹配对照，血友病 A 是否与 TSA 后更多的出血和血栓栓塞不良事件相关？ (2) 与匹配对照相比，A 型血友病患者的 5 年 TSA 假体生存率是否会降低？方法 使用 2010 年至 2022 年 PearlDiver M161 数据库来识别接受初次解剖或反向 TSA 的患者。鉴于 X 连锁隐性血友病 A 几乎全部出现在男性中，接受 TSA 的男性血友病 A 患者与接受 TSA 的无血友病男性患者根据年龄和 Elixhauser 合并症指数 (ECI) 以 1:10 进行匹配。 73 名接受 TSA 的 A 型血友病患者与 729 名非血友病患者以 1:10 的比例进行匹配。将九十天的不良事件与多变量分析进行比较。使用 Kaplan-Meier 分析评估 5 年内的修订情况。结果 与对照组相比，血友病患者出现出血问题的几率更大（血肿，OR 6.8 [95% CI 3.0 - 15.3]；p < 0.001；贫血，OR 2.5 [95% CI 1.5 - 4.2]；p % 3C 0.001，输血，OR 5.0 [95% CI 2.4 至 10.3]；p < 0.001)，静脉血栓栓塞事件 (VTE)（OR 1.9 [95% CI 1.1 至 3.1]；p = 0.01），以及假体松动 (OR 3.5 [95% CI 1.4 至 8.0]；p = 0.004）。根据现有数据，血友病患者的 5 年植入物存活率 (97.3% [95% CI 93.6% to 100.0%]) 与匹配对照组 (95.2% [95% CI 93.4% to 97.2%]) 没有差异；p = 0.60）。 结论 血友病患者 90 天出血并发症（血肿、贫血和输血）和 VTE（DVT 和 PE）的风险均升高，强调了在术前、术中和术后仔细平衡因子替代和 VTE 预防的特殊挑战在血液科医生的仔细协调下，以个体患者为基础。有必要进一步研究该人群中因子 VIII 的水平和目标以及氨甲环酸和 VTE 预防，以提供进一步的指导。此外，根据现有数据，血友病患者和匹配对照（无血友病患者）之间的 5 年植入物存活率没有差异，这表明 TSA 存活率仍然持久，并且可以按照指示向该人群中的患者提供。证据级别 III 级，治疗研究。