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Diagnosis and surgical treatment for isolated tricuspid libman-sacks endocarditis: a rare case report and literatures review
© Zhixuan et al. 2015
Received: 6 October 2014
Accepted: 23 June 2015
Published: 8 July 2015
Libman-Sacks endocarditis (LSE), characterized by verrucous vegetations formation, is a typical cardiac manifestation of autoimmune diseases such as systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). It primarily leads to lesions of cardiac valves and mostly involved valves are mitral and aortic, but isolated tricuspid valve involvement is exceptional. Here we reported a 20-years-old female with past SLE history suffered from acute right heart failure caused by multiple tricuspid vegetations and valve regurgitation. The patient recovered following tricuspid valve replacement with a bioprosthesis. Transesophageal echocardiography(TEE), especially real time 3-dimensional (RT3D) TEE provide a better imaging modality for assessing cardiac valvular involvement of LSE. For patients with active SLE/APS course, uncontrolled systemic inflammation may made it difficult for surgical exposure and suture. The durability of bioprosthesis for this patient and the prosthesis selection for tricuspid LSE both need further follow-up and more clinical investigation.
KeywordsEndocarditis Three dimesional transesophageal echocardiography Tricuspid valve prosthesis Tricuspid regurgitation Systemic lupus erythematosus
In 1924, Libman and Sacks originally reported 4 systemic lupus erythematosus (SLE) cases with verrucose vegetative endocarditis , and that was the first introduction of Libman-Sacks endocarditis(LSE). Nowadays, LSE have been seen as a typical cardiac manifestation of autoimmune diseases such as SLE and antiphospholipid syndrome(APS). The pathologic changes of LSE involve the formation of fibrin-platelet thrombi on the altered valve, the organization of which leads to valve fibrosis, edema, diffuse thickening, mild inflammatory changes, valve distortion, scarring, and subsequent valvular dysfunction such like stenosis or regurgitation [2, 3]. LSE often involve left heart valves, tricuspid lesions were very rare. And most LSE can be treated with medicine therapy while very few need surgical treatment. Here we reported a rare tricuspid LSE patient with SLE history which underwent open-heart surgery with bioprosthesis replacement.
A 20-year-old young Asian female presented to our department for continuous weakness and short of breath for more than 2 months on June 2014. The girl’s past history was significant for systemic lupus erythematosus (SLE) more than 3 years but she denied regular oral hormone therapy for nearly 2 years. Symptoms of paroxysmal knee joint ache, paroxysmal nocturnal dyspnea and orthopnea are manifested. She had experienced increased dyspnea on exertion with activities of daily living and increasing lower extremity edema.
The verrucous vegetations formation of LSE are seen as a cardiac manifestation of SLE and/or APS. Mitral and aortic valve is the mostly involved valve, while tricuspid valve involvement was rarely reported. Moyssakis reported 38 LSE in 342 SLE patient which were diagnosed by echocardiography, among which there were 24 mitral and 13 aortic involvement. only one tricuspid involvement . Doppler echocardiography can be considered as the diagnostic technique of choice. But sometimes it is very difficult to identify LSE and true infectious endocarditis (IE), for the former may also have fever due to the original immunology diseases and the latter may also have vegetation. Echocardiographically, LSE vegetations appear as masses of varying size and shape with irregular borders and heterogeneous echo-density, which are firmly attached to the leaflet surface and exhibit no independent motion . While the vegetation of IE may typically exhibit independent motion . The role of transesophageal echocardiography (TEE), especially RT3D TEE, had been emphasized in assessing vegetation size in a patient with LSE . And in our case the diagnostic role of RT3D TEE had been also highlighted, which might be more sensitive to the very special verrucous vegetations.
Treatment for LSE consists of drug treatment and surgical intervention. Corticosteroid and anticoagulation drugs are used for LSE drug treatment. Corticosteroids cannot prevent LSE, but they can help healing LSE lesions by lessening inflammation [3, 7, 8]. However they can also increase tissue fibrosis and scarring, finally worsening valvular damage and dysfunction. Nonetheless, appropriate and sufficient steroid therapy to control autoimmune disease activity is important. Anticoagulation therapy is required due to the increased risk of thrombo-embolic events in LSE and current therapeutic guidelines for APS did suggest long-term anticoagulation to prevent thrombo-embolic events [2, 3]. So if the patient with LSE is hemodynamically stable, conservative treatments above should be firstly recommended. Moaref had reported a successfully recovered case of LSE with treatment of prednisolone and hydroxychloroquine, but the patient denied worsening symptoms of heart failure and received drug treatments by months . If with severe intractable symptomatic valvular dysfunction, surgical intervention for LSE may be required [10, 11]. Since our patient got symptoms of acute uncontrolled right heart failure, early surgical intervention even without adequate steroid treatment should be considered.
Reviews of literature on isolated tricuspid valve surgery for TS/TR caused by Libman-Sacks endocarditis
SLE and/or APS
Adequate steroids usage before surgery
Heart failure before surgery
Laufer et al. 
Dilated annulus and elongated chordae, no vegetations
Mechanical prosthesis replacement
Ledingham et al. a
a large calcified mass invading the right side of the heart
Subsequent tricuspid stenosisafter initial surgery
Removal but no re-implantation of tricuspid prothesis
Alive 5 years post-2ndop
Chan-Lam et al. a
two masses that were adherent to the tricuspid valve and intermittently prolapsed through the pulmonary valve.
Surgical removal of the masses
Falode et al. 
massive vegetations involving the tricuspid valve, filling the right atrium
Vegetation removal and valve replacement (type of prothesis not mentioned)
Alive 3 months post-op
Gur et al. 
multiple verrucous vegetations as if being a mass on the anterior and posterior leaflets
Tricuspid valve commisurotomy and Kay annuloplasty
Alive 6 months post-op
Wang et al. 
several large nodules on the subvalvular apparatus and the atrial side of the leaflets of tricuspid valve; small perforation on a septal leaflet
Mechanical valve replacement
Alive 5 weeks post-op
Bai et al.
A large and mutiple tiny vegetations on the atrial side of anterior leaflet with ruptured main chordae tendinae of the leaflet
Alive 2 months post-op
Usually for LSE, it was not very recommended to implant a bioprosthesis since there was reported cases that underwent re-operation in the future, due to rapid calcification, valvulitis and subsequent perforation  or massive bioprosthetic thrombosis . So a mechanical prosthesis might provide a comprehensive better result for LSE. But there were no expert suggestion on prosthesis selection for tricuspid site for LSE. For normal consideration, mechanical tricuspid valve replacement (TVR) leads to increased early mortality  and occurrence of valve-related events, especially the composite of thrombosis, embolism, and bleeding . Compared to the possibility of mid-to-long-term degeneration and failure of bioprosthesis, we could not tell which kind of prosthesis on tricuspid site should have better outcomes. Considering the prosthesis site and the gender/age of our patient, a mechanical valve may be not very recommended since it might need more intense anticoagulation at tricuspid site which may lead to unexpected embolism-bleeding events and bring harassments for female menstruation and pregnancy. But the durability of bioprosthesis for this patient and the prosthesis selection for tricuspid LSE both need further follow-up and more clinical investigation.
LSE should be strongly suspected when significant valve vegeation unveiled during the course of SLE and/or APS. Mitral involvement is common but tricuspid LSE is rarely reported. TEE, especially RT3D-TEE, is useful for diagnosis between LSE and IE. Conservative treatment with steroids should be firstly recommended, but patients with untreated and severe intractable symptomatic valvular dysfunction still need surgical intervention. For patients with active SLE/APS course, uncontrolled systemic inflammation may made it difficult for surgical exposure and suture. The prosthesis selection for tricuspid LSE when valve repair is impossible, mechanical or bioprothesis, need further follow-ups and more clinical cases investigation.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
This study was financially supported by Department of Cardiovasular Surgery, West China Hospital of Sichuan University.
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