Left atrial thrombi have been considered to be the major source of systemic arterial embolization in patients with rheumatic mitral valve disease. Almost half of the left atrial thrombi are found in the left atrial appendage (LAA). To investigate LAA size and LAA contractile function in patients with rheumatic mitral valve disease, transesophageal echocardiographic and Doppler studies were performed in 61 patients. Among them, 46 patients were in atrial fibrillation (group 1), while the other 15 were in sinus rhythm (group 2). Thirty-six patients with nonrheumatic atrial fibrillation were chosen as control to group 1. Another 22 patients with various cardiovascular diseases and sinus rhythm served as control to group 2. When compared to the patients with nonrheumatic atrial fibrillation (control group), group 1 patients tended to have a larger LAA maximal area (9.7 ± 5.2 vs. 5.9 ± 2.8 cm²; p < 0.001). LAA ejection fraction and LAA peak emptying velocity were also lower. A significantly higher incidence of LAA spontaneous echo contrast (SEC) and thrombus formation was also found in these patients. Group 2 patients were also found to have a larger LAA maximal area when compared to the control group (8.8 ± 3.7 vs. 5.2 ± 3.0 cm²; p < 0.001). LAA ejection fraction and LAA peak emptying velocity were lower in this group, too. A higher incidence of LAA SEC formation was found in these patients with rheumatic mitral valve disease (4/15 vs. 0/22; p = 0.021). There was no significant difference, however, in LAA thrombus formation between group 2 and its control group (1/15 vs. 1/22; p = NS). Thus, patients with rheumatic mitral valve disease tend to have larger LAA size and poorer LAA contractile function. These promote the formation of LAA SEC and thrombus, especially when the patients have atrial fibrillation. Whether the poor LAA function predisposes these patients to future systemic arterial embolization still needs further investigation.