Prevalence, Morphologic Types, and Evolution of Cardiac Valvular Disease in Systemic Lupus Erythematosus

Abstract

We performed echocardiography prospectively 4.9 +/- 0.7 years apart (mean +/- SD), in 74 patients with systemic lupus erythematosus. On the basis of the first study, the patients were distributed in four groups according to the type of valvular involvement: 7 patients had vegetations (Libman-Sacks endocarditis; group 1); 6 patients had rigid and thickened valves with stenosis, regurgitation, or both (group 2); 5 patients had miscellaneous forms of valvular involvement without valvular dysfunction (group 3), as did the 60 controls; and 56 patients had no valvular disease (group 4). The overall prevalence of clinically important valvular disease (groups 1 and 2) was 18 percent. Patients in group 1 were younger than those in group 2 (33.5 +/- 16.7 vs. 47.8 +/- 17.6 years; P less than 0.05), had a shorter mean duration of lupus (4.8 +/- 2.2 vs. 10.7 +/- 6.4 years; P less than 0.001), and had received a smaller cumulative dose of steroids (21.5 +/- 13.1 vs. 79.5 +/- 63.4 g of methylprednisolone or its equivalent; P less than 0.05). During the five-year follow-up, one patient in group 1 and five in group 2 required valve surgery, no patient in group 3 had valvular dysfunction, and five patients in group 4 had mild valvular lesions. We conclude that clinically important valvular involvement in systemic lupus is relatively frequent and sometimes requires surgery. Echocardiography can identify a subset of lesions (valvular thickening and dysfunction), other than verrucous (Libman-Sacks) endocarditis, that are prone to hemodynamic deterioration.

Autologous hematopoietic stem cell transplantation in systemic lupus erythematosus patients with cardiac dysfunction: feasibility and reversibility of ventricular and valvular dysfunction with transplant-induced remission

“…This is not a surprising finding since correlation between severity of lupus and presence of cardiac involvement has not been demonstrated in other studies. 13,15 The potential cardiac toxicity of both autologous and allogeneic HSCT in patients with hematological malignancies and cardiac co-morbidities has been reported for decades, 5,16,17 with poor pre-transplant LVEF being associated with a higher incidence of cardiac morbidity and mortality in some studies. 18 As a result, patients with hematological malignancies and coexistent significant cardiac dysfunction are precluded from transplant.…”

“…13 The etiology of valve dysfunction in SLE is likely related to endocardial inflammation with LibmanSacks endocarditis. Interestingly, no data to date support treating lupus endocarditis with immunosuppression, and hence the mechanism of observed response to auto-HSCT among our patients with valvular dysfunction is uncertain.…”

Severe aortic stenosis and mitral regurgitation in a woman with systemic lupus erythematosus

“…Valvular disease is the most characteristic and clinically important form of cardiac lesions. 1,2 Echocardiography has identified two types of valvular lesions with a different prognostic significance. 2 The most specific damage is characterized by the presence of nonbacterial verrucous vegetations that were first described by Libman and Sacks and are usually associated with mild valvular regurgitation.…”

Cardiac Valve Diseases and Antiphospholipid Syndrome

“…Those data suggest that the presence of aPLs represents a risk for having valvular involvement in patients with SLE and/or APS (8). The pathogenesis of Libman-Sacks endocarditis has been hypothesized to involve fibrin thrombi on the altered valve and its organization leads to fibrosis and dysfunction (9). aPLs may play a role to mediate valvular damage by promoting thrombin formation on the endothelium (8).…”

“…However, considering the immunomediated mechanism in aPL related valvular involvement, the inflammatory reaction in the affected valves may be suppressed by steroids (17). On the other hand, scarring and deformity of the valve after steroid administration mayultimately lead to valve dysfunction (9).…”