Valvular disease associated with systemic illness

The connective tissue diseases are immune-mediated inflammatory diseases that manifest predominantly with symptoms and signs of musculoskeletal and mucocutaneous inflammation. They frequently affect the heart valves, pericardium, and myocardium. In patients with AKS, the aortic root and conduction system are also frequently involved. Echocardiographic series in these patients have demonstrated that valvular disease is highly prevalent and associated with substantial morbidity and mortality (Table 1). The prevalence rates of clinically detected valvular disease, however, are either unknown or low. This discrepancy is related to lack of awareness, overshadowing of the cardiovascular manifestations by the inflammatory symptoms and signs of the musculoskeletal system, lack of systematic application of the history and cardiovascular physical examination, and high sensitivity of echocardiography for detecting subclinical abnormalities. Several valvular abnormalities have been identified as unique to a specific disease. Libman-Sacks vegetations, valve nodules, and subaortic bump are characteristic of SLE, RA, and AKS (see Table 1). The valvular complications and respective therapy are similar to those of other causes of valvular disease; however, the associated morbidity and mortality of these complications in these patients are high. The worse prognosis of valvular disease in these patients is related to the chronicity and debilitating nature of their illness, their high prevalence of multisystem disease, and immunosuppression. These factors underscore the importance of early recognition, prevention of complications, and proper clinical or echocardiographic follow-up. The distinctive echocardiographic characteristics of the valve abnormalities associated with the connective tissue diseases may allow their differentiation from other common valvulopathies, such as infective endocarditis, rheumatic valvular disease, and degenerative valvular disease (Table 2). Despite the clinical and prognostic implications of valvular disease associated with the connective tissue diseases, incomplete data are available about pathogenesis, relation to clinical features of the primary disease, evolution, and effect of steroid or cytotoxic therapy. Echocardiography, especially TEE, has the potential to redefine the prevalence rates and to characterize better the valve abnormalities associated with these conditions. Finally, future large cross-sectional and longitudinal studies using clinical and echocardiographic data may help to define better the presence, evolution, and therapy of the valvular disease associated with the connective tissue diseases.