Sat0197 non Myocardial Cardiac Involvement in Antiphospholipid Syndrome in a Spanish Reference Center

Abstract

Background:Antiphospholipid syndrome (APS) is a systemic autoimmune disease, associated with a hypercoagulable state and fetal loss and with other clinical manifestations including cardiac involvement. APS occurs as a primary disorder (PAPS) or secondary to another autoimmune disease (SAPS). Due to its vascular nature, various organs and tissues may be affected, including the cardiac system. Cardiac manifestations of APS are valve abnormalities (valve thickening and vegetations), occlusive arterial disease (atherosclerosis and myocardial infarction) and pulmonary hypertension (PH).Objectives:To assess the prevalence of non-myocardial involvement (valvulopathy and pulmonary hypertension) in a cohort of patients with antiphospholipid antibodies (aPLs).Methods:Retrospective observational study in a Spanish reference center for systemic autoimmune diseases. All patients with aPLs and performed transthoracic echocardiogram (TTE) were included in the study. Patients were divided between PAPS, SAPS and aPLs carriers. A cohort of 50 patients with systemic lupus erythematosus (SLE) without aPLs was used as a control. Anti-cardiolipin, anti-B2GP1 and lupus anticoagulant antibodies were determined by standard techniques.Results:A total of 220 patients were reviewed. 145 (65.9%) were female. The mean age was 42 years. Among all patients with aPLs, 102 were PAPS, 73 SAPS, and 45 asymptomatic carriers (silent APS). Patients with aPLs, unlike patients with SLE without aPLs, presented more often pathological TTE (114 patients, 52%) (p = 0.02), with more valvular involvement (87, 39%) (p = 0.005) and pulmonary hypertension (21, 9.5%, p = ns). Valve involvement was identified in 99 patients: 45 in PAPS, 27 in SAPS, 14 in aPLs carriers and 13 in the SLE without aPLs, these differences being statistically significant (p = 0.002). Valvulopathy was asymptomatic in the majority of patients but required valve replacement in two patients. Mitral valve was the most affected, especially in the form of insufficiency (57%), followed by aortic valve, combined mitral and aortic valve, and less frequently the pulmonary valve alone (3 cases).aPLs globalPAPSSAPSaPLs carriersSLE w/o aPLsTotal220 (100%)102 (46.4%)73 (33.2%)45 (20.4%)50 (100%)Men75 (34.1%)41 (40.2%)23 (31.5%)11 (24.4%)6 (12%)Women145 (65.9%)61 (59.8%)50 (68.5%)34 (75.6%)44 (88%)Conclusion:Subclinical valve involvement was very common in patients with APS. There was no correlation with other clinical manifestations of APS nor were other risk factors identified. PH was less frequent than valvular involvement in patients with APS. However, despite not being statistically significant, close to 10% of patients with APS had PH compared to 6% of patients without APS.aPLs globalPAPSSAPSaPLs carriersSLE w/o aPLsTTE performed220 (100%)102 (46.4%)73 (33.2%)45 (20.4%)50 (100%)Pathological TTE114 (52%)16 (32%)p=0.02Valvular involvement87 (39%)45 (20.4%)27 (12.3%)14 (6.3%)13 (26%)p=0.005Pulmonary hypertension21 (9.5%)3 (6%)p=nsEvery patient with APS should have an echocardiogram in the initial study protocol in order to rule out both valvulopathy and pulmonary hypertension. This could modify the patient’s management both in the short and long term, as well as the prognosis.References:[1]Radin M, Ugolini-Lopes MR, Sciascia S, Andrade D. Extra-criteria manifestations of antiphospholipid syndrome: Risk assessment and management.Semin Arthritis Rheum. 2018;48(1):117–120.[2]Tenedios F, Erkan D, Lockshin MD. Cardiac involvement in the antiphospholipid syndrome.Lupus. 2005;14(9):691–696.[3]Kolitz T, Shiber S, Sharabi I, Winder A, Zandman-Goddard G. Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form.Front Immunol. 2019;10:941.Disclosure of Interests:None declared