Mitral Valve Replacement for Libman-Sacks Endocarditis in Antiphospholipid Syndrome Secondary to Systemic Lupus Erythematosus Complicated with Thrombocytopenic Purpura

(Division of Cardiovascular Surgery, Toyota Kosei Hospital, Toyota, Japan and Division of Thoracic and Cardiovascular Surgery, Nagoya Ekisaikai Hospital*, Nagoya, Japan)

Masaharu Yoshikawa Osamu Kawaguchi Akira Takanohashi*
Kei Yagami* Fumiaki Kuwabara* Yuichi Hirate*
Yuichi Hirate* , Yoshiya Miyata*
A 42-year-old woman with antiphospholipid syndrome(APLS)secondary to systemic lupus erythematosus(SLE)complicated with thrombocytopenic purpura was successfully treated by mitral valve replacement with a mechanical prosthesis and tricuspid valve annuloplasty for mitral valve stenosis and regurgitation due to Libman-Sacks endocarditis. Intraoperative hemorrhagic oozing due to thrombocytopenia was effectively managed with platelet transfusion. Negative microbial culture and pathological examination of the resected mitral valve demonstrated an atypical sterile verrucose lesion, the findings of which were typically characteristic of Libman-Sacks endocarditis in SLE. She was successfully discharged 31 days after the operation without any hemorrhagic or thromboembolic events. However, 100 days after surgery, she suffered from fatal cerebral infarction caused by poor Coumadin compliance. Regarding the prosthetic valve selection, it is reasonable to select the mechanical valve because 1)anticoagulation therapy is necessary for APLS, 2)the risk of the dialysis induction due to the lupus-induced renal failure leading to a high calcium turnover, which results in accelerated bioprosthetic valve calcification. In case of SLE with APLS, in which anticoagulation and antiplatelet therapy is required to prevent the thromboembolic event and thrombocytopenic purpura, after valve replacement, strict management of anticoagulation plays an essential role to prevent thromboembolic complication.
  Jpn. J. Cardiovasc. Surg. 38:67-70(2009)