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.
 Perez-Villa F Font J Azqueta M Espinosa G Pare C Cervera R et al. Severe valvular regurgitation and antiphospholipid antibodies in systemic lupus erythematosus: a prospective long-term follow up study. Arthritis Rheum 2005;53:460–7.
 Gabrielli F Alcini E di Prima MA Mazzacurati G Masala C. Cardiac valve involvement in systemic lupus erythematosus and primary antiphospholipid syndrome: lack of correlation with antiphospholipid antibodies. Int J Cardiol 1995;51:117–26.
 Borowski A Ghodsizad A Cohnen M Gams E. Recurrent embolism in the course of marantic endocarditis. Ann Thorac Surg 2005;79:2145–7.
 Eiken PW Edwards WD Tazelaar HD McBane RD Zehr KJ. Surgical pathology of nonbacterial thrombotic endocarditis in 30 patients 1985–2000. Mayo Clin Proc 2001;76:1204–12.
 Moyssakis I Tzioufas A Triposkiadis F Strateges N Kyriakidis M. Severe aortic stenosis and mitral regurgitation in a woman with systemic lupus erythematosus. Clin Cardiol 2002;4:194–6.
 Muscal E Brey RL. Neurologic manifestations of the antiphospholipid syndrome: integrating molecular and clinical lessons. Curr Rheumatol Rep 2008;10:67–73.
 Denas G Jose SP Bracco A Zoppellaro G Pengo V. Antiphospholipid syndrome and the heart: a case series and literature review. Autoimmun Rev 2015;14:214–22.
 Jouhikainen T Pohjola-Sintonen S Stephansson E. Lupus anticoagulant and cardiac manifestations in systemic lupus erythematosus. Lupus 1994;3:167–72.
 Leung WH Wong KL Lau CP Wong CK Liu HW. Association between antiphospholipid antibodies and cardiac abnormalities in patients with systemic lupus erythematosus. Am J Med 1990;89:411–9.
 Espínola-Zavaleta N Vargas-Barrón J Colmenares-Galvis T Cruz-Cruz F Romero-Cárdenas A Keirns C et al. Echocardiographic evaluation of patients with primary antiphospholipid syndrome. Am Heart J 1999;137:973–8.
 Hojnik M George J Ziporen L Shoenfeld Y. Heart valve involvement (Libman–Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93:1579–87.
 Morelli S Bernardo ML Viganego F Sgreccia A De Marzio P Conti F et al. Left-sided heart valve abnormalities and risk of ischemic cerebrovascular accidents in patients with systemic lupus erythematosus. Lupus 2003;12:805–12.
 Roldan CA Shively BK Crawford MH. An echocardiographic study of valvular heart disease associated with systemic lupus erythematosus. N Engl J Med 1996;335:1424–30.
 Turiel M Sarzi-Puttini P Peretti R Bonizzato S Muzzupappa S Atzeni F et al. Five-year follow-up by transesophageal echocardiographic studies in primary antiphospholipid syndrome. Am J Cardiol 2005;96:574–9.
 Dentali F Manfredi E Crowther M Ageno W. Longduration therapy with low molecular weight heparin in patients with antiphospholipid antibody syndrome resistant to warfarin therapy. J Thromb Haemost 2005;3:2121–3.
 Sciascia S Khamashta MA D’Cruz DP. Targeted therapy in antiphospholipid syndrome. Curr Opin Rheumatol 2014;26:269–75.
 Bakshi J Stevens R. Rituximab therapy for recurrent thromboembolic disease in antiphospholipid syndrome. Lupus 2013;22:865–7.
 Dufrost V Risse J Zuily S Wahl D. Direct Oral Anticoagulants Use in Antiphospholipid Syndrome: Are These Drugs an Effective and Safe Alternative to Warfarin? A Systematic Review of the Literature. Curr Rheumatol Rep 2016;18:74.
 Cohen H Hunt BJ Efthymiou M Arachchillage DJ Mackie IJ Clawson S et al. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome with or without systemic lupus erythematosus (RAPS): a randomized controlled open-label phase 2/3 non-inferiority trial. Lancet Haematol 2016;3:426–36.
 Nesher G Ilany J Rosenmann D Abraham AS. Valvular dysfunction in antiphospholipid syndrome: prevalence clinical features and treatment. Semin Arthritis Rheum 1997;27:27–35.
 Erdozain JG Ruiz-Irastorza G Segura MI Amigo MC Espinosa G Pomar JL et al. Cardiac Valve Replacement in Patients with Antiphospholipid Syndrome. Arthritis Care Res (Hoboken) 2012;64:1256–60.
 Gorki H Malinovski V Stanbridge RD. The antiphospholipid syndrome and heart valve surgery. Eur J Cardiothorac Surg 2008;33:168–81.