抗磷脂综合征并发严重的Libman-Sacks心内膜炎：23例手术病例的回顾性分析

Severe Libman-Sacks endocarditis complicating antiphospholipid syndrome: a retrospective analysis of 23 operated cases.

作者信息

Chalvon Nellie Bourse, Costedoat-Chalumeau Nathalie, Pennaforte Jean-Loup, Servettaz Amelie, Boulagnon Rombi Camille, Gavand Pierre-Edouard, Lekieffre Maud, Le Guern Veronique, Morel Nathalie, Cohen Aubart Fleur, Haroche Julien, Mathian Alexis, Collet Jean-Philippe, Piette Jean-Charles, Amoura Zahir, Orquevaux Pauline

机构信息

Department of Internal Medicine, University Hospital Center Reims, Reims.

Department of Internal Medicine, Faculté Paris 6, Paris.

出版信息

Rheumatology (Oxford). 2023 Feb 1;62(2):707-715. doi: 10.1093/rheumatology/keac315.

DOI:10.1093/rheumatology/keac315

PMID:35686908

Abstract

OBJECTIVE

Data on severe heart valve disease (HVD), including Libman-Sacks endocarditis, associated with SLE and/or APS requiring valvular surgery are scarce. We thus conducted a retrospective study, aimed at describing and clarifying clinical, laboratory, echocardiographic, histopathological and evolutional features of SLE and/or APS patients with severe associated-HVD.

METHODS

An observational retrospective multicentric analysis of 23 adults with SLE and/or APS and HVD between 1996 and 2019 and available histopathological report evaluating long-term follow-up.

RESULTS

Twenty-three individuals (20 females, median age 37 [range 17-76] years) were included. All had APS (thrombotic in 22, with an arterial phenotype in 15 and with catastrophic APS [CAPS] in six), and 11 (47%) had SLE. Systemic underlying disease had been diagnosed prior to HVD in 12 (52%). In 10 patients (43%), HVD was complicated by cerebral stroke prior to surgery. Twenty patients (87%) had only one pathological valve, the mitral valve in 18 patients (78%). Valvular thickening (n = 19) and valvular regurgitation (n = 19) were the most frequently reported lesions. Fifteen (62%) patients underwent mechanical valve replacement, six (26%) conservative valve repair (five were later re-operated after a median time of 1 [0-4] year), and two (9%) underwent biological valve replacement. Nine patients (39%) presented early-onset post-operative complications, including three CAPS immediately after surgery and one death. After surgery, 18 patients (78%) had normal postoperative valvular function, but almost half of the patients (43%) had post-operative neurological sequelae (median follow-up of 6 [2-20] years).

CONCLUSION

Severe HVD leading to surgery was strongly associated with thrombotic APS, especially arterial phenotypes. Half of the reported patients presented cerebral stroke complicating the HVD. Valvular surgery carried a significant risk of CAPS.

摘要

目的

关于严重心脏瓣膜病（HVD），包括与系统性红斑狼疮（SLE）和/或抗磷脂综合征（APS）相关的Libman - Sacks心内膜炎且需要进行瓣膜手术的数据稀缺。因此，我们开展了一项回顾性研究，旨在描述和阐明患有严重相关HVD的SLE和/或APS患者的临床、实验室、超声心动图、组织病理学及病情演变特征。

方法

对1996年至2019年间23例患有SLE和/或APS及HVD的成年人进行一项观察性回顾性多中心分析，并获取评估长期随访情况的组织病理学报告。

结果

纳入23例个体（20例女性，中位年龄37岁[范围17 - 76岁]）。所有患者均患有APS（22例为血栓形成型，其中15例为动脉型，6例为灾难性抗磷脂综合征[CAPS]），11例（47%）患有SLE。12例（52%）患者在HVD之前已诊断出全身性基础疾病。10例患者（43%）在手术前HVD并发脑卒。20例患者（87%）仅有一个病变瓣膜，其中18例（78%）为二尖瓣。瓣膜增厚（n = 19）和瓣膜反流（n = 19）是最常报告的病变情况。15例（62%）患者接受了机械瓣膜置换，6例（26%）接受了保守瓣膜修复（其中5例在中位时间1年[0 - 4年]后再次手术），2例（9%）接受了生物瓣膜置换。9例患者（39%）出现早期术后并发症，包括术后立即发生3例CAPS和1例死亡。术后，18例患者（78%）瓣膜功能正常，但几乎一半的患者（43%）有术后神经后遗症（中位随访时间6年[2 - 20年]）。