Behcet's Disease as a Frequently Unrecognized Cause of Aortic Regurgitation: Suggestive and Misleading Echocardiography Findings

Background: Behcet's disease (BD) is a systemic vasculitis that can be a possible cause of aortic regurgitation (AR). When aortic valve replacement is performed in patients with BD, unless preoperative immunosuppressive therapy and special operative techniques are used, the postoperative course is usually complicated by valve dehiscence, necessitating another operation. The preoperative diagnosis of BD is challenging because of its protean manifestations, but an early diagnosis is associated with a more favorable prognosis. Methods: Between 1990 and 2008, 15 patients with final diagnoses of BD underwent aortic valve replacement. Medical records and echocardiographic findings were retrospectively analyzed. Results: Of the 15 patients, only 2 were diagnosed as having BD prior to first operation. Twelve underwent repeat open-heart surgery because of valve dehiscence, and 6 of these 12 patients eventually died. In 5 patients with available echocardiographic findings of native valve morphology, echocardiography demonstrated characteristic aneurysmal changes and redundant coronary cusp motion in 4 patients. Vegetation-like mobile lesions and echo-free spaces mimicking abscess pockets led to misdiagnoses of infective endocarditis in 7 patients at least once during their clinical courses. Furthermore, complete atrioventricular block requiring permanent pacemaker insertion developed in 5 patients, especially those with echo-free spaces in the periannular area. Conclusions: Dismal prognoses are almost inevitable unless etiologic diagnoses are made prior to surgery in patients with BD and AR. It is important to diagnose BD preoperatively, and the diagnostic importance of echocardiographic findings should be kept in mind when evaluating patients with AR. (J Am Soc Echocardiogr 2009;22:1269-74.)