A case report of an unusual cause of mitral stenosis in a young woman

Abstract

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease, frequently associated with cardiovascular involvement. One of the most frequent complication is mitral valve regurgitation in more than one-third of the patients.

Case summary: A 30-year-old woman with arthralgia, butterfly rash, and Raynaud phenomenon presented with a systolic murmur and renal impairment. Based on the kidney biopsy the diagnosis of SLE was made. Echocardiography revealed the presence of pulmonary hypertension, restrictive mitral valve disease with nodular thickening of the anterior leaflet and moderate regurgitation, compatible with Libman Sacks (LS) endocarditis. Immunosuppressive therapy was started and the patient status improved with normalization of systolic pulmonary artery pressure. After 8 years without follow-up, she presented with fatigue and dyspnoea based on a severe mitral valve stenosis. Subsequently, she underwent a minimal invasive mitral valve replacement and the diagnosis of LS endocarditis could be confirmed upon histopathological examination.

Discussion: This case demonstrates that LS endocarditis can not only lead to mitral regurgitation but occasionally to mitral stenosis due to chronic inflammation with thickening and fusion of mitral valve leaflets. Hereby, comprehensive echocardiography, inclusive stress echocardiography, plays a critical role.

Keywords: Case report; Libman Sacks endocarditis; Mitral stenosis; Stress echocardiography; Systemic lupus erythematosus.

Figure 1

Two- and three-dimensional rest echocardiographical images from 2006 (baseline) to 2015 (follow-up) in a patient with systemic lupus erythematosus and progressive Libman Sacks endocarditis. (A, B, D, and E) Mitral valve area by planimetry of three- and two-dimensional planes in short axis are shown. (F) Reduced maximal diastolic opening (also see Supplementary material online, Video S2) with nodular thickening in 2015 compared with 2006 (C, see Supplementary material online, Video S3) on an apical four-chamber view is shown.

Figure 2

Bicycle echocardiography in 2015 at rest and during peak stress (100 W). Images show tricuspid regurgitant flow velocities (A and C) and continuous wave Doppler mitral velocities (B and D). Mean mitral valve gradient became much higher at peak stress (mean 34 vs. 6 mmHg) (D vs. B) with a significant increase in systolic pulmonary pressure (67 mmHg compared with 40 mmHg at rest) (C vs. A).

Figure 3

The excised mitral valve macroscopic view (A) shows the chordae tendineae (*) and mitral valve. Microscopic examination (B) shows the presence of distrophic calcifications (**) and strong widened collagen connective tissue and hyalinization. (C) neovascularization (typical for lupus) and chronic inflammation with plasma cells infiltration is shown. Haematoxylin and eosin stain, original magnification at ×10 (A), ×50 (B), and ×310 (C).