We present a case of probably unreported aortic stenosis evolution in a patient with primary antiphospholipid syndrome (APS). A female patient, 54 years old, with a history of recurrent deep venous thrombosis, an episode of pulmonary embolism and was positive for APS antibodies. She was kept on warfarin and aspirin. The patient was admitted with acute pulmonary edema and severe aortic stenosis. While preparing for aortic valve surgery, the patient developed acute stroke, and a week later developed concurrent acute ischemia of both lower limbs. Emergency surgery salvaged the lower limbs and follow up transthoracic echocardiography showed marked regression of the thickening of the aortic valve leaflets, only mild aortic stenosis and moderate aortic regurgitation. Aortic valve surgery was cancelled, and the patient was kept on warfarin and aspirin.
Development of severe aortic stenosis is uncommon in primary APS, and this scenario of spontaneous improvement from a severe to a mild aortic stenosis, is probably unreported before.