Double-valve Replacement for Mitral and Aortic Regurgitation in a Patient with Libman-Sacks Endocarditis

Hachiya, Kenta; Wakami, Kazuaki; Tani, Tohru; Yoshida, Atsuhiro; Suzuki, Shugo; Suda, Hisao; Ohte, Nobuyuki

doi:10.2169/internalmedicine.53.2232

Cited by 11 publications

(8 citation statements)

References 7 publications

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“…Case reports suggest that native valve repair does not alter the progression of valve thickening and calcification, and that replacement is ultimately necessary. 2 Additionally, it has been reported that while corticosteroid therapy improves overall survival of patients with SLE, their use may cause shrinking and scarring of heart valves. 3 It has also been noted that porcine bioprosthetic valves can be affected by valvulitis with perforation in the cusps in patients with SLE requiring replacement.…”

Section: Discussionmentioning

confidence: 99%

Aortic valve replacement for Libman-Sacks endocarditis

Keenan

Janardhanan²,

Larsen

et al. 2016

BMJ Case Reports

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A 24-year-old man with systemic lupus erythematosus and antiphospholipid syndrome complicated by lupus nephritis presented with acute limb ischaemia secondary to an embolus. Following embolectomy, the patient underwent a transthoracic echocardiogram which revealed a large vegetation on all three cusps of the aortic valve. The patient was taken for an urgent aortic valve replacement with a mechanical valve. Cultures of one cusp remained sterile. Histopathological examination of the remaining two cusps revealed sterile fibrin-rich thrombotic vegetations characteristic of non-bacterial thrombotic endocarditis.

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Section: Discussionmentioning

confidence: 99%

Aortic valve replacement for Libman-Sacks endocarditis

Keenan

Janardhanan²,

Larsen

et al. 2016

BMJ Case Reports

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“…The mortality risk of surgical valve replacement is higher in patients with SLE and especially in patients under immunosuppressive regimen [19]. In fact, among the two patients who underwent surgery in our study, death occurred in the subject receiving high doses of steroids.…”

Section: Discussionmentioning

confidence: 60%

Endocarditis in Systemic Lupus Erythematosus

Kâaroud¹,

Bezzine²,

Hamida³

et al. 2017

IJSM

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Endocarditis is one of the most prevalent forms of cardiac involvement in patients with lupus, as it considered as a life-threatening complication. The aim of this study is to determine particularities of endocarditis in patients with lupus and to look for distinguishing features between infectious or immunological origin. A retrospective study was conducted on patients with lupus presenting endocarditis. Lupus diagnosed according to the American College of Rheumatology criteria. The diagnosis of endocarditis was made based on the modified Duke criteria. The present case report studies seven cases of endocarditis. Six of these patients are women, and the other one is a man. They are aged meanly of 29.4 years (extremes: 20-36). Fever was present in all the cases with a new cardiac murmur in six cases and a modification of its intensity in one instance. The biologic inflammatory syndrome was present in six cases. A cardiac ultrasound performed in six cases made the diagnosis of endocarditis which involved the left heart valves in five cases and the right heart valves in one instance. Valvular insufficiency was identified in six patients. The valve involvement was mitral in two cases, mitral-aortic in two others, aortic in the fifth one and tricuspid in the sixth one. Endocarditis was infectious in 4 cases, thanks to positive blood culture. The germs identified were gram-negative bacilli in two cases, the anaerobic organism in one instance and gram-positive cocci in one instance. Candida albicans was isolated in one case. Libman-Sacks endocarditis was objectified in three cases. A combination of Libman-Sacks endocarditis with infectious endocarditis was diagnosed in one instance. The treatment consisted of antibiotics in four cases with surgery in two cases. The outcome was favorable in five cases and fatal in the two others. Endocarditis in lupus can be infectious or Libman-Sacks endocarditis. These two conditions share several clinical features. The only distinctive argument remains positive blood culture. The treatment should be initiated as early as possible to limit the valve damage and improve the outcome.

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“…While appropriate immunosuppressive therapy is essential to control SLE disease activity, corticosteroid therapy for Libman–Sacks-related valvular dysfunction reportedly results in increased fibrosis and scarring, followed by worsened valvular damage and dysfunction 6 , 9 . Furthermore, in part due to mineralocorticoid effects, worsening MR with decompensated heart failure may occur after high-dose corticosteroid therapy 5 , 13 . In our patient, MR decreased from severe to mild after immunosuppressive therapy including corticosteroids.…”

Section: Discussionmentioning

confidence: 63%

“…Libman–Sacks-related valvular dysfunction ranges from a variable degree of inflammatory cell infiltration along with oedematous changes and fibrin deposits to end stage or healed forms with a fibrous plaque. The degree of progression is associated with SLE duration and activity 5 . The pathogenesis involves the formation of fibrin–platelet thrombi due to inflammation, which organize and lead to fibrosis and scarring with subsequent valve dysfunction over time 12 …”

Section: Discussionmentioning

confidence: 99%

“…The clinical presentation of Libman–Sacks valvular changes ranges from asymptomatic valvular thickening with oedematous changes to severe valvular dysfunction 3 , 4 . Improvement in Libman–Sacks endocarditis-related mitral regurgitation (MR) after surgical repair or replacement has been documented 5 , 6 ; however, to the best of our knowledge, the effectiveness of immunosuppressive therapy against these changes has not been reported. We describe a case of severe MR due to Libman–Sacks endocarditis that improved with immunosuppressive therapy.…”

Section: Introductionmentioning

confidence: 99%

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Immunosuppressive therapy to reduce mitral regurgitation in Libman–Sacks endocarditis: a case report

Ishizu

Isotani

Yamaji

et al. 2019

European Heart Journal - Case Reports

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Background Libman–Sacks endocarditis is a cardiac manifestation of systemic lupus erythematosus (SLE) and is characterized by non-bacterial verrucous vegetations, causing valvular stenosis and/or regurgitation. The effectiveness of immunosuppressive therapy for valve dysfunction due to Libman–Sacks endocarditis has not been reported. Case summary A 67-year-old woman with a history of chronic atrial fibrillation was emergently admitted with acute decompensated heart failure. Transoesophageal echocardiogram revealed severe mitral regurgitation (MR) due to oedematous thickening and poor coaptation of the medial edge of A2/P2 segments and the lateral edge of A3/P3 segments. Serial blood culture results were negative, suggesting bacterial infective endocarditis to be a less likely cause of valvular damage. Because the patient developed photosensitivity, livedo reticularis, and pancytopenia, Libman–Sacks endocarditis with rapidly progressive SLE was diagnosed on the basis of positive test results of anti-double-stranded DNA-IgG and its complement titer. Two months after, immunosuppressive therapy including corticosteroids, a transoesophageal echocardiogram revealed thinning of the degenerative mitral valve leaflets and a reduction of MR from severe to mild. Discussion Corticosteroid therapy for Libman–Sacks endocarditis reportedly increases the extent of fibrosis and scarring of the valve leaflets, resulting in worse valve function. In our patient, MR decreased from severe to mild after corticosteroid therapy. Because low-echoic thickening of the mitral valve leaflets suggested acute oedematous changes without scarring and fibrosis and other clinical symptoms of SLE rapidly progressed, early initiation of immunosuppressive therapy for Libman–Sacks endocarditis lead to a benign clinical course in our patient.

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Double-valve Replacement for Mitral and Aortic Regurgitation in a Patient with Libman-Sacks Endocarditis

Cited by 11 publications

References 7 publications

Aortic valve replacement for Libman-Sacks endocarditis

Aortic valve replacement for Libman-Sacks endocarditis

Endocarditis in Systemic Lupus Erythematosus

Immunosuppressive therapy to reduce mitral regurgitation in Libman–Sacks endocarditis: a case report

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