Abstract
INTRODUCTION: Antiphospholipid syndrome (APS) is a multisystem autoimmune disorder of hypercoagulability, characterized by arterial and venous thrombosis, recurrent fetal losses, thrombocytopenia, and circulating antiphospholipid antibodies. Among valvular manifestations associated with APS, mitral regurgitation (MR) is the most common, followed by aortic regurgitation. Surgical interventions for APS-related valvular diseases carry high perioperative morbidity and mortality. There are no established guidelines regarding the surgical management of APS-related MR. CASE PRESENTATION: A 46-year-old man underwent mitral valve repair for severe MR. Histopathological examination of the vegetation on the mitral valve revealed nonbacterial thrombotic endocarditis, and in combination with laboratory findings, the MR was diagnosed as associated with APS. Leaflet thickening and fusion progressed postoperatively, leading to moderate-to-severe MR and mitral stenosis within 2 years. CONCLUSIONS: In cases where MR is the initial presentation of APS, preoperative diagnosis of APS can be challenging. Detailed, frame-by-frame transesophageal echocardiographic evaluation may aid in the preoperative identification of subtle valvular abnormalities suggestive of APS. When thrombocytopenia and a prolonged activated partial thromboplastin time (APTT) are identified during preoperative evaluation for valvular heart disease, tests for lupus anticoagulant, anticardiolipin antibody, and anti-β2 glycoprotein I antibody should be performed, and consultation with hematologists is recommended. Valve repair for MR associated with APS, which preserves valvular tissue that may become diseased in the future, is likely to result in poor outcomes.