Jaccoud's arthropathy (JA) was initially described as a secondary complication to rheumatic fever (RF). However, most recently described cases are associated with systemic lupus erythematosus (SLE). At least in RF, this articular complication has been observed to occur in association with valvular heart disease. The aim of this work is to investigate the presence of valvulopathy in patients with SLE and JA, when compared to lupus patients without such complication. Patients with diagnosis of SLE based on the American College of Rheumatology criteria were enrolled in the study and divided into two groups: with or without JA and evaluated by transthoracic echocardiography. A total of 113 patients with SLE (25 with JA and 88 without JA) were assessed, of which 108 were females and five were males. Echocardiographic changes were found in 24 patients (21.2%) out of the entire population, including valvulopathy in 17 cases (15%), pulmonary hypertension in 7 cases (6.2%) and pericardial effusion in 2 cases (1.8%). In general, echocardiographic changes were more frequently seen in the JA group in comparison with the control group (p = 0.04). Additionally, in the JA group, valvulopathy was found in nine cases (36%) against eight cases (9%) in the control group (p = 0.001). This study reveals for the first time the association between the presence of valvular heart disease and JA in SLE patients, suggesting that the presence of JA may be a marker of such complication. Additional studies are required for clarification of the mechanisms involved in both complications.