Severe Aortic Stenosis and Dual Valve Vegetations: An Uncommon Presentation of Primary Antiphospholipid Syndrome.

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.