Senesael Ellie, Plein Danièle, La Meir Mark, Droogmans Steven
Department of Cardiology, University Hospital of Ghent, C.Heymanslaan 10, Ghent, Belgium.
Department of Cardiology, Centre for Cardiovascular Diseases, University Hospital of Brussels, Laarbeeklaan 101, Jette, Belgium.
Eur Heart J Case Rep. 2018 Oct 30;2(4):yty118. doi: 10.1093/ehjcr/yty118. eCollection 2018 Dec.
Systemic lupus erythematosus (SLE) is an autoimmune disease, frequently associated with cardiovascular involvement. One of the most frequent complication is mitral valve regurgitation in more than one-third of the patients.
A 30-year-old woman with arthralgia, butterfly rash, and Raynaud phenomenon presented with a systolic murmur and renal impairment. Based on the kidney biopsy the diagnosis of SLE was made. Echocardiography revealed the presence of pulmonary hypertension, restrictive mitral valve disease with nodular thickening of the anterior leaflet and moderate regurgitation, compatible with Libman Sacks (LS) endocarditis. Immunosuppressive therapy was started and the patient status improved with normalization of systolic pulmonary artery pressure. After 8 years without follow-up, she presented with fatigue and dyspnoea based on a severe mitral valve stenosis. Subsequently, she underwent a minimal invasive mitral valve replacement and the diagnosis of LS endocarditis could be confirmed upon histopathological examination.
This case demonstrates that LS endocarditis can not only lead to mitral regurgitation but occasionally to mitral stenosis due to chronic inflammation with thickening and fusion of mitral valve leaflets. Hereby, comprehensive echocardiography, inclusive stress echocardiography, plays a critical role.
系统性红斑狼疮（SLE）是一种自身免疫性疾病，常伴有心血管受累。最常见的并发症之一是超过三分之一的患者出现二尖瓣反流。
一名30岁女性，有关节痛、蝶形红斑和雷诺现象，伴有收缩期杂音和肾功能损害。根据肾脏活检确诊为SLE。超声心动图显示存在肺动脉高压、二尖瓣狭窄伴前叶结节状增厚及中度反流，符合Libman-Sacks（LS）心内膜炎。开始免疫抑制治疗后，患者病情改善，收缩期肺动脉压恢复正常。在失访8年后，她因严重二尖瓣狭窄出现疲劳和呼吸困难。随后，她接受了微创二尖瓣置换术，组织病理学检查证实为LS心内膜炎。
该病例表明，LS心内膜炎不仅可导致二尖瓣反流，还可因二尖瓣叶增厚和融合的慢性炎症偶尔导致二尖瓣狭窄。因此，全面的超声心动图检查，包括负荷超声心动图，起着关键作用。
