Abstract 4147071: Lupus Mitral Valve Disease Masquerading as an Absent Posterior Mitral Valve Leaflet

Introduction/Background: Marantic endocarditis and valvulitis are cardiovascular manifestations of autoimmune disease that can be challenging to diagnose. Case Presentation: A 23-year-old female with SLE presented with brief episodes of intermittent left eye vision loss and left sided weakness. She also reported fevers, a malar rash, and new onset dyspnea on exertion. Serologic work-up was consistent with an SLE flair. MRI of the brain/orbits demonstrated two acute infarcts in the frontal and parieto-occipital lobes. CTA head and neck did not show any large vessel vasculitis. Transthoracic echocardiography showed thickening of the mitral valve (MV) leaflets, with moderate MR and an elevated MV gradient of 11 mm Hg. The posterior leaflet was not visualized, concerning for absent or hypoplastic posterior leaflet. A transesophageal echocardiogram was performed and showed severe thickening of posterior mitral leaflet with restricted motion and shortening secondary to extensive thrombus burden. In addition, there was evidence of thrombus at the tip of the anterior MV leaflet. The patient was managed with high dose steroids and warfarin for SLE flair and marantic endocarditis, respectively. Two months later, she presented with amaurosis fugax. Cardiac MRI showed thickened MV leaflets with delayed enhancement along the posterior leaflet and MV annulus consistent with an organized thrombus. After multidisciplinary discussion, the patient underwent surgical MV replacement with a bioprosthetic valve. Intraoperative evaluation revealed severely thickened MV leaflets with fused commissures. In addition, there was fusion of the posterior leaflet and lateral side of the anterior leaflet to the papillary muscles as a result of thickened and shortened chordae. These findings were believed to be due to inflammatory changes involving the MV apparatus. Surgical pathology confirmed the diagnosis of valvulitis. The patient has done well post operatively. Conclusion: Valvulitis and marantic endocarditis can have devastating consequences if not treated promptly. Use of multimodality imaging can aid in the diagnosis. Multidisciplinary discussion is critical in achieving desired clinical outcomes.