Diagnostic value of transesophageal echocardiography in Libman-Sacks endocarditis

Libman-Sacks endocarditis is the most common cardiac manifestation in patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (PAPS); it is characterized by Libman-Sacks vegetations, valve thickening or fibrosis, valve regurgitation, and rarely valve stenosis. It is most commonly clinically manifested with cardioembolism (predominantly to the brain), uncommonly with symptomatic severe valve regurgitation, or rarely with over imposed infective endocarditis. These three clinical syndromes are associated with a five to eight times higher morbidity and mortality than in a general population. Libman-Sacks endocarditis is infrequently detected by the history and cardiovascular physical examination; it is detected by transthoracic echocardiography (TTE) in 30-40% as compared to 60-80% of patients by transesophageal echocardiography (TEE). Also, TTE as compared to TEE has low sensitivity (63% overall, 11% for valve vegetations), low specificity (58%), low negative predictive value (40%), and a moderate positive predictive value (78%) for the detection of Libman-Sacks endocarditis. In addition, TEE has played a major role in our current understanding of the specific characteristics, evolution, assessment of prognosis, and defining the need and response to medical or surgical therapy of Libman-Sacks endocarditis. Therefore, an increased awareness of the potential clinical complications of Libman-Sacks endocarditis and application of echocardiography, especially of TEE, may lead to an earlier and accurate diagnosis, guidance of therapy, prevention of complications, decreased rate of progression of the disease, and consequently to an event free survival of patients with SLE or PAPS.