系统性红斑狼疮及抗磷脂综合征相关心脏瓣膜病的外科治疗与转归

Surgical Treatment and Outcomes of Systemic Lupus Erythematosus and Antiphospholipid Syndrome-Related Valvular Heart Disease

摘要: 目的：研究因系统性红斑狼疮(Systemic lupus erythematosus, SLE)及SLE合并抗磷脂抗体综合征(Antiphospholipid-antibody syndrome, APS)患者心脏瓣膜病变(Heart Valve Disease, HVD)行外科手术治疗患者的临床特征、围术期管理及预后，以明确影响术后结局的关键因素，为优化此类高危患者的治疗策略提供依据。方法：通过单中心回顾性队列研究，纳入2017年1月至2023年12月青岛大学附属医院因SLE/APS相关HVD行心脏瓣膜手术的10例患者。收集基线特征(如年龄、性别、SLEDAI评分、APS状态)、围术期管理(激素/免疫抑制剂使用、抗凝策略)、手术方式(生物瓣/机械瓣置换)及术后结局(瓣膜功能、并发症、生存率)。通过随访(中位2.64年)评估瓣膜结构稳定性及患者转归。结果：10例患者进入本研究，男、女性各5例，男性5例，确诊SLE时的中位年龄在59.5岁，4例患者在心脏瓣膜手术前已确诊SLE；3例患者原发病SLE与心脏瓣膜病一同确诊，3例术后随访期间初始诊断为SLE。5例SLE患者(50.0%)同时合并抗磷脂抗体综合征，术前SLEDAI评分为7.5 (6~12)分。瓣膜病变最常累及二尖瓣(70%)，其次是主动脉瓣(30%)，3例患有Libman-Sacks心内膜炎，并引起瓣膜关闭不全。所有患者均行心脏瓣膜置换术，1例更换机械瓣，9例更换生物瓣。术后早期3例(37.5%)死亡，死因均与狼疮剧烈活动引发严重并发症有关。其余7人，3例(43%)患者于术后晚期发生并发症，2例患者发生脑梗死，1例患者发生急性左心衰竭、心房纤颤、急性肾功能衰竭及置换的人工瓣膜破坏。经过平均2.64年的随访后，7例患者中(10例患者中去掉早期死亡的3例)，2例患者再次出现置换瓣膜的结构及功能破坏，1例患者原未经置换的自身瓣膜进展为严重反流。结论：SLE、APS患者可出现严重的心脏瓣膜疾病，可表现为无菌性赘生物(Libman-Sacks心内膜炎)、瓣叶增厚、瓣膜反流和瓣膜狭窄，可导致严重瓣膜功能不全，术后并发症及死亡率很高，均与原发病活动有关，原发病加重及恶化会导致术后瓣膜失功，术前明确SLE/APS诊断、术后强化免疫抑制及抗凝治疗是改善预后的关键。因此心脏外科医师术前需要明确导致瓣膜改变的SLE原发病，持续关注并精准治疗才有可能达到最终的治疗目的。

Abstract: Objective: To investigate the clinical characteristics, perioperative management, and prognosis of patients undergoing surgical treatment for heart valve disease (HVD) associated with systemic lupus erythematosus (SLE) or SLE combined with antiphospholipid syndrome (APS), aiming to identify key factors influencing postoperative outcomes and optimize treatment strategies for these high-risk patients. Methods: A single-center retrospective cohort study included 10 patients who underwent cardiac valve surgery at the Affiliated Hospital of Qingdao University between January 2017 and December 2023 for SLE/APS-associated HVD. Data on baseline characteristics (age, sex, SLEDAI score, APS status), perioperative management (glucocorticoid/immunosuppressant use, anticoagulation strategy), surgical approach (bioprosthetic/mechanical valve replacement), and postoperative outcomes (valve function, complications, survival) were collected. Valve structural stability and patient prognosis were assessed during a median follow-up of 2.64 years. Results: The cohort comprised 5 males and 5 females, with a median age of 59.5 years at SLE diagnosis. Four patients were diagnosed with SLE before surgery, 3 concurrently with HVD, and 3 during postoperative follow-up. Five SLE patients (50.0%) had coexisting APS, with a median preoperative SLEDAI score of 7.5 (range: 6~12). The mitral valve (70%) and aortic valve (30%) were most frequently affected. Three patients exhibited Libman-Sacks endocarditis, causing valvular regurgitation. All patients underwent valve replacement (9 bioprosthetic, 1 mechanical). Early postoperative mortality was 37.5% (3/10), all due to severe lupus-related complications. Among the 7 survivors, 3 (43%) developed late complications: cerebral infarction (2 cases), and acute left heart failure, atrial fibrillation, acute renal failure, with bioprosthetic valve dysfunction (1 case). During follow-up, 2 patients showed structural/functional deterioration of replaced valves, and 1 developed severe regurgitation in a native non-replaced valve. Conclusions: Patients with SLE and APS can develop severe valvular heart disease, manifesting as aseptic vegetations (Libman-Sacks endocarditis), leaflet thickening, valvular regurgitation, and valvular stenosis, leading to severe valvular insufficiency. Postoperative complications and mortality are high, all related to the activity of the primary disease. Exacerbation and deterioration of the primary disease can lead to postoperative valvular failure. Preoperative diagnosis of SLE/APS and postoperative intensive immunosuppression and anticoagulation therapy are key to improving prognosis. Therefore, cardiac surgeons need to identify the underlying SLE causing valvular changes preoperatively and continuously monitor and precisely treat it to achieve the ultimate therapeutic goal.