Background/Purpose: Valvular disease represents the most common cardiac manifestation among patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) positive for antiphospholipid antibodies (aPL). Diastolic dysfunction of the left and right ventricle has also been observed in these groups of patients. The purpose of the present study is to describe the evolution of valve involvement and myocardial dysfunction over time in patients with SLE and/or APS and investigate possible associations with clinical and laboratory characteristics.

Methods: One hundrend and fifty patients had been assessed by transthoracic echocardiography 10 years ago. Structural and functional (regurgitation or stenosis) valvular abnormalities and diastolic function parameters ( ‘E’ and ‘A’ waves, E to A ratio, deceleration time (DT), isovolumic relaxation time (IVRT)) for the left and right ventricle had initially been recorded. The longitudinal arm of the study finally included 17 patients with primary APS (PAPS), 23 with SLE-asscociated APS (SLE/APS), 19 with SLE positive for aPL without APS and 23 with SLE negative for aPL, for the present echocardiographic re-evaluation. 

Results: The proportion of patients with valvulopathy increased from 39.3% in the initial cohort to 64.7% among PAPS and 61.5% among SLE patients. Worsening of valvular lesions during the 10 year follow-up period was detected in approximately one third of the examined population including PAPS and SLE patients, either positive or negative for aPL. Disease duration and presence of SLE/APS were the only significant risk factors for progression of isolated mitral and combined valvular disease; there was an 1.5 times higher risk for progression for every 5 years of increase in disease duration (OR:1.54, 95% C.I:1.05-2.25, p=0.027 and OR:1.63, 95% C.I: 1.13-2.36, p=0.009, respectively) and 3.5 times higher risk for SLE/APS patients (OR:3.57, 95% C.I:1.19-10.70, p=0.023 and OR:3.51, 95% C.I:1.27-9.67, p=0.015, respectively). Presence of aPL, aPL titres, presence of comorbidities and other clinical characteristics did not have any significant effect on progression of valvular lesions. No treatment regimen seemed to have a prophylactic role in preventing  occurrence of de novo valvular lesions or in halting progression of preexisting valvulopathy. Left ventricular diastolic dysfunction similarly progressed over time with DT and IVRT being equally prolonged in each of the 4 groups (p<0.05). Right ventricular DT was significantly prolonged in each of the 3 SLE groups (p<0.001), but IVRT increased only in SLE/APS patients (p=0.040).

Conclusion: Progression of valvulopathy, as detected by transthoracic echocardiography, was observed in the majority of patients with either SLE or APS, despite treatment of underlying disease. Secondary APS in SLE and disease duration were independent risk factors for progression of valvular disease. Ventricular diastolic dysfunction, primarily of the left ventricle, similarly progressed over the 10 year period.

 

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/myocardial-dysfunction-and-valvulopathy-worsens-with-time-in-patients-with-antiphospholipid-syndrome-a-10-year-follow-up-study/