Mitral valve surgery for mitral regurgitation caused by Libman-Sacks endocarditis: a report of four cases and a systematic review of the literature

Wobbe Bouma,Theo J Klinkenberg,Massimo A Mariani,Felix Zijlstra,Albert Jh Suurmeijer,Michiel E Erasmus,Marc Bijl,Iwan Cc Van Der Horst,Inez J Wijdh-Den Hamer

doi:10.1186/1749-8090-5-13

Wobbe Bouma, Theo J Klinkenberg + Show 7 more

Open Access

https://doi.org/10.1186/1749-8090-5-13

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Abstract

Libman-Sacks endocarditis of the mitral valve was first described by Libman and Sacks in 1924. Currently, the sterile verrucous vegetative lesions seen in Libman-Sacks endocarditis are regarded as a cardiac manifestation of both systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). Although typically mild and asymptomatic, complications of Libman-Sacks endocarditis may include superimposed bacterial endocarditis, thromboembolic events, and severe valvular regurgitation and/or stenosis requiring surgery. In this study we report two cases of mitral valve repair and two cases of mitral valve replacement for mitral regurgitation (MR) caused by Libman-Sacks endocarditis. In addition, we provide a systematic review of the English literature on mitral valve surgery for MR caused by Libman-Sacks endocarditis. This report shows that mitral valve repair is feasible and effective in young patients with relatively stable SLE and/or APS and only localized mitral valve abnormalities caused by Libman-Sacks endocarditis. Both clinical and echocardiographic follow-up after repair show excellent mid- and long-term results.

Highlights

In 1924 Libman and Sacks first described four cases of non-bacterial verrucous vegetative endocarditis [1]
This report shows that mitral valve repair is feasible and effective in young patients with relatively stable systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS) and only localized mitral valve abnormalities caused by Libman-Sacks endocarditis
In this study we report two cases of mitral valve repair and two cases of mitral valve replacement for mitral regurgitation (MR) caused by LS endocarditis

Summary

Introduction

In 1924 Libman and Sacks first described four cases of non-bacterial verrucous vegetative endocarditis [1]. The sterile verrucous lesions of Libman-Sacks (LS) endocarditis (Fig 1) show a clear predisposition for the mitral and aortic valves and are nowadays seen as both a cardiac manifestation of systemic lupus erythematosus (SLE) and, more recently, of the antiphospholipid syndrome (APS) [2,3,4,5]. Over the last decades with prolonged survival and improvement in diagnostic techniques, in echocardiography, cardiac disease associated with SLE has become more apparent [6,7]. A recent echocardiographic study in patients with SLE revealed that LS vegetations can be found in approximately 11% of patients with SLE [8].

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