LAUSR

INTRODUCTION Data on severe heart valve disease (HVD), including Libman-sacks endocarditis, associated with systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS) requiring valvular surgery are scarce. METHOD Observational retrospective multicentric analysis of 23 adults with SLE and/or APS and HVD, between 1996 and 2019 and available histopathological report evaluating long-term follow-up. RESULTS Twenty-three individuals (20 females, median age 37 [range 17-76]) were included. All had APS (thrombotic in 22, with an arterial phenotype in 15 and with catastrophic APS (CAPS) in 6), and 11 (47%) had SLE. Systemic underlying disease had been diagnosed prior to HVD in 12 (52%). In 10 patients (43%), HVD was complicated by cerebral stroke prior to surgery. Twenty patients (87%) had only one pathological valve, the mitral valve in 18 patients (78%). Valvular thickening (n = 19) and valvular regurgitation (n = 19) were the most frequently reported lesions. Fifteen (62%) patients underwent mechanical valve replacement, six (26%) conservative valve repair (five were later re-operated after a median time of 1 [0-4] years), and two (9%) underwent biological valve replacement. Nine patients (39%) presented early-onset post-operative complications, including three CAPS immediately after surgery and one death. After surgery, 18 patients (78%) had normal postoperative valvular function, however almost half of the patients (43%) had post-operative neurological sequelae (median follow-up of 6 [2-20] years). CONCLUSION Severe HVD leading to surgery was strongly associated with thrombotic APS, especially arterial phenotypes. Half of the reported patients presented cerebral stroke complicating the HVD. Valvular surgery carried a significant risk of CAPS.