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Libman-Sacks Endocarditis in a Patient With Antiphospholipid Syndrome
Libman-Sacks Endocarditis in a Patient With Antiphospholipid Syndrome Kunal D. Kotkar, MS, MCH, and Sameh M. Said, MD Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota
Antiphospholipid syndrome is a systemic autoimmune syndrome with cardiac manifestations such as nonbacterial thrombotic endocarditis, also known as LibmanSacks endocarditis. A 61-year-old female with history of antiphospholipid syndrome presented in acute pulmonary edema. Echocardiography demonstrated mobile vegetations on the free margins of both the anterior and the posterior mitral valve leaflets. Blood cultures and fungal serologies were negative. During mitral valve replacement both the anterior and posterior mitral leaflets were covered with multiple small vegetations with features of nonbacterial thrombotic endocarditis. Though mostly asymptomatic, Libman-Sacks endocarditis may be an indication for valve replacement. (Ann Thorac Surg 2016;102:e31–2) Ó 2016 by The Society of Thoracic Surgeons
A
ntiphospholipid syndrome (aPLs) is a systemic autoimmune syndrome characterized by arterial and venous thrombosis, recurrent fetal losses, and persistently elevated levels of antiphospholipid antibodies [1]. This syndrome may be primary when it is unassociated with any other autoimmune disorders or may be secondary when associated with disorders such as systemic lupus erythematosus (SLE). Cardiac manifestations of the syndrome include nonbacterial thrombotic endocarditis (NBTE), also known as Libman-Sacks endocarditis, and other valvular manifestations such as valve thickening and dysfunction. We present a case of a middle-aged female with aPLs and Libman-Sacks endocarditis who presented with acute pulmonary edema.
Fig 1. Intraoperative photo showing a view of the left atrium with the classical wart-like vegetations of Libman-Sacks endocarditis involving both the anterior and posterior mitral leaflets.
Decision was made to proceed with mitral valve replacement. Intraoperatively, through a standard left atriotomy, the mitral valve was examined and both the anterior and posterior mitral leaflets were covered with multiple small vegetations that were all located at the free margins of the leaflets and were consistent with features of NBTE (Figs 1, 2). Both leaflets were excised and mitral valve replacement with 29 mm SJM mechanical prosthesis (St. Jude Medical, St. Paul, Minnesota) was performed. Postoperative recovery was uneventful and patient was dismissed on postoperative day 5. Pathological examination of excised valve leaflets demonstrated NBTE with no evidence of microorganisms.
Comment Libman-Sacks endocarditis was originally described by Emanuel Libman and Benjamin Sacks in 1924 in 4 patients with atypical sterile verrucous lesions of the valvular and mural endocardium [2]. The lesions were
A 61-year-old female with history of aPLs presented with severe dyspnea (New York Heart Association functional class IV) and hemoptysis. Chest x-ray showed pulmonary edema-like picture with patchy ground glass opacities and non-specific intralobar septal thickening. Transthoracic echocardiography demonstrated severe mitral valve stenosis. Transesophageal echocardiography showed moderate to severe mitral regurgitation, severe mitral stenosis with a mean diastolic gradient of 12 mm Hg, and thickened, mobile vegetations on the free margins of both the anterior and the posterior mitral valve leaflets. Blood cultures and fungal serologies were sent and were all negative.
Accepted for publication Nov 2, 2015. Address correspondence to Dr Said, Mayo Clinic, 200 1st St SW, Rochester, MN 55905; email: [email protected].
Ó 2016 by The Society of Thoracic Surgeons Published by Elsevier
Fig 2. Photograph of the excised mitral valve showing the classical wart-like vegetation of Libman-Sacks endocarditis involving the anterior and posterior leaflets. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.11.004
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CASE REPORT KOTKAR AND SAID LIBMAN-SACKS ENDOCARDITIS
believed to be characteristic of SLE. The association between Libman-Sacks endocarditis and antiphospholipid antibodies was first noted in 1985 in a young woman with SLE and lupus anticoagulant [3]. Libman-Sacks valvular lesions are sterile fibrofibrinous vegetations that may develop anywhere on the endocardial surface of the heart but with a propensity for the left valves, particularly the ventricular surface of the mitral valve. They are typically sessile, wart-like, and small. Similar verrucous valvular lesions have been identified on valves from patients with aPLs, either primary or secondary to SLE. On echocardiography, vegetations appeared as valve masses of varying size and shape with irregular borders and echodensity, firmly attached to the valve surface and exhibiting no independent motion [4]. The pathogenesis of NBTE involves the formation of fibrin-platelet thrombi on altered valve leading to fibrosis, distortion of valve architecture, and eventual valvular dysfunction.
Ann Thorac Surg 2016;102:e31–2
In the majority of patients, valvular involvement is of minor hemodynamic significance and does not cause clinically significant heart disease. However, in some patients the valve may be sufficiently damaged to cause heart failure requiring valve replacement, as was illustrated in our case [4].
References 1. Djokovic A, Stojanovic L. [Cardiac manifestations in antiphospholipid syndrome–a brief review of the literature]. Srp Arh Celok Lek 2015;143:346–53. 2. Libman E, Sacks B. A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 1924;33: 701–37. 3. D’Alton JG, Preston DN, Bormanis J, Green MS, Kraag GR. Multiple transient ischemic attacks, lupus anticoagulant and verrucous endocarditis. Stroke 1985;16:512–4. 4. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93:1579–87.
Antiphospholipid syndrome is a systemic autoimmune syndrome with cardiac manifestations such as nonbacterial thrombotic endocarditis, also known as LibmanSacks endocarditis. A 61-year-old female with history of antiphospholipid syndrome presented in acute pulmonary edema. Echocardiography demonstrated mobile vegetations on the free margins of both the anterior and the posterior mitral valve leaflets. Blood cultures and fungal serologies were negative. During mitral valve replacement both the anterior and posterior mitral leaflets were covered with multiple small vegetations with features of nonbacterial thrombotic endocarditis. Though mostly asymptomatic, Libman-Sacks endocarditis may be an indication for valve replacement. (Ann Thorac Surg 2016;102:e31–2) Ó 2016 by The Society of Thoracic Surgeons
A
ntiphospholipid syndrome (aPLs) is a systemic autoimmune syndrome characterized by arterial and venous thrombosis, recurrent fetal losses, and persistently elevated levels of antiphospholipid antibodies [1]. This syndrome may be primary when it is unassociated with any other autoimmune disorders or may be secondary when associated with disorders such as systemic lupus erythematosus (SLE). Cardiac manifestations of the syndrome include nonbacterial thrombotic endocarditis (NBTE), also known as Libman-Sacks endocarditis, and other valvular manifestations such as valve thickening and dysfunction. We present a case of a middle-aged female with aPLs and Libman-Sacks endocarditis who presented with acute pulmonary edema.
Fig 1. Intraoperative photo showing a view of the left atrium with the classical wart-like vegetations of Libman-Sacks endocarditis involving both the anterior and posterior mitral leaflets.
Decision was made to proceed with mitral valve replacement. Intraoperatively, through a standard left atriotomy, the mitral valve was examined and both the anterior and posterior mitral leaflets were covered with multiple small vegetations that were all located at the free margins of the leaflets and were consistent with features of NBTE (Figs 1, 2). Both leaflets were excised and mitral valve replacement with 29 mm SJM mechanical prosthesis (St. Jude Medical, St. Paul, Minnesota) was performed. Postoperative recovery was uneventful and patient was dismissed on postoperative day 5. Pathological examination of excised valve leaflets demonstrated NBTE with no evidence of microorganisms.
Comment Libman-Sacks endocarditis was originally described by Emanuel Libman and Benjamin Sacks in 1924 in 4 patients with atypical sterile verrucous lesions of the valvular and mural endocardium [2]. The lesions were
A 61-year-old female with history of aPLs presented with severe dyspnea (New York Heart Association functional class IV) and hemoptysis. Chest x-ray showed pulmonary edema-like picture with patchy ground glass opacities and non-specific intralobar septal thickening. Transthoracic echocardiography demonstrated severe mitral valve stenosis. Transesophageal echocardiography showed moderate to severe mitral regurgitation, severe mitral stenosis with a mean diastolic gradient of 12 mm Hg, and thickened, mobile vegetations on the free margins of both the anterior and the posterior mitral valve leaflets. Blood cultures and fungal serologies were sent and were all negative.
Accepted for publication Nov 2, 2015. Address correspondence to Dr Said, Mayo Clinic, 200 1st St SW, Rochester, MN 55905; email: [email protected].
Ó 2016 by The Society of Thoracic Surgeons Published by Elsevier
Fig 2. Photograph of the excised mitral valve showing the classical wart-like vegetation of Libman-Sacks endocarditis involving the anterior and posterior leaflets. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.11.004
e32
CASE REPORT KOTKAR AND SAID LIBMAN-SACKS ENDOCARDITIS
believed to be characteristic of SLE. The association between Libman-Sacks endocarditis and antiphospholipid antibodies was first noted in 1985 in a young woman with SLE and lupus anticoagulant [3]. Libman-Sacks valvular lesions are sterile fibrofibrinous vegetations that may develop anywhere on the endocardial surface of the heart but with a propensity for the left valves, particularly the ventricular surface of the mitral valve. They are typically sessile, wart-like, and small. Similar verrucous valvular lesions have been identified on valves from patients with aPLs, either primary or secondary to SLE. On echocardiography, vegetations appeared as valve masses of varying size and shape with irregular borders and echodensity, firmly attached to the valve surface and exhibiting no independent motion [4]. The pathogenesis of NBTE involves the formation of fibrin-platelet thrombi on altered valve leading to fibrosis, distortion of valve architecture, and eventual valvular dysfunction.
Ann Thorac Surg 2016;102:e31–2
In the majority of patients, valvular involvement is of minor hemodynamic significance and does not cause clinically significant heart disease. However, in some patients the valve may be sufficiently damaged to cause heart failure requiring valve replacement, as was illustrated in our case [4].
References 1. Djokovic A, Stojanovic L. [Cardiac manifestations in antiphospholipid syndrome–a brief review of the literature]. Srp Arh Celok Lek 2015;143:346–53. 2. Libman E, Sacks B. A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 1924;33: 701–37. 3. D’Alton JG, Preston DN, Bormanis J, Green MS, Kraag GR. Multiple transient ischemic attacks, lupus anticoagulant and verrucous endocarditis. Stroke 1985;16:512–4. 4. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996;93:1579–87.