JACC. Case reports, 10 2 2025, Pages 106227 Severe Aortic Stenosis and Dual Valve Vegetations: An Uncommon Presentation of Primary Antiphospholipid Syndrome. van der Woude SFS, Valentijn FA, Antoni ML, Huisman SJ, van Vliet M
Background
Primary antiphospholipid syndrome (PAPS) may cause thromboembolic events and valvular heart disease.
Case summary
A 42-year-old woman with a history of pre-eclampsia, epilepsy, and thrombocytopenia presented with acute chest pain and shortness of breath. Investigations revealed vegetation on both the aortic and mitral valves, severe aortic stenosis, and a non-ST-segment elevation myocardial infarction. Autoimmune testing confirmed antiphospholipid antibodies, which led to a diagnosis of PAPS with Libman-Sacks endocarditis, as well as secondary coronary embolic complications. After multidisciplinary consultation, the patient received anticoagulation, immunosuppressive therapy, and plasmapheresis.
Discussion
This case report underlines the aggressive nature of PAPS, particularly given its presentation of severe aortic stenosis and dual valve involvement in a relatively young patient. To our knowledge, this is the first reported case of such a presentation.
Take-home message
PAPS can manifest with fulminant cardiac manifestations including severe aortic stenosis, dual valve vegetations, and myocardial infarction, emphasizing the importance of early recognition and management.
