ABSTRACT
Patients with Antiphospholipid syndrome (APLS) are at high risk for both bleeding and thrombotic complications during cardiac surgery involving cardiopulmonary bypass (CPB). In this case we present a patient with APLS and Immune Thrombocytopenic Purpura who successfully underwent aortic valve replacement (AVR) with CPB despite recent craniotomy for subdural hematoma evacuation. Anticoagulation for CPB was monitored by targeting an Activated Clotting Time (ACT) that was 2× the upper limit of normal. A multidisciplinary approach was essential in ensuring a safe and successful operation.
ABSTRACT
Objective:To analyze the clinical characteristics and risk factors of systemic lupus erythematosus (SLE) with Libman-Sacks endocarditis (LSE).Methods:Data of SLE patients with LSE ( n=20) who admitted in Peking Union Medical College Hospital from January 2012 to May 2021 were retrospectively collected. SLE patients without LSE ( n=60) were randomly selected as controls according to 1∶3 age and sex matched in the hospitalized patients during the same period. Clinical characteristics, laboratory and imaging examinations were analyzed. Data were expressed as Mean± SD, and t test was used to compare quantitative data in normal distri-bution. Data were expressed as M ( Q1, Q 3), and Wilcoxon signed-rank test or Wilcoxon rank sum test were used to compare quantitative data in non-normal distribution. The count data were compared with Chi-square test or Fisher's exact test. Univariate conditional logistic regression was used for univariable analyses. P values less than 0.05 were considered statistically significant. Results:There were 20 SLE patients with LSE, 18 females with an average age of (32±9) years (13 to 49 years). The disease duration of SLE was 16.04(0, 185.1) months when LSE was discovered, and vegetations were located at the mitral valve in 19 (95.0%) patients. Cerebral infarction (45.0% vs 10.0%, χ2=9.87, P=0.001) and antiphospholipid syndrome (APS) (50.0% vs 5.0%, χ2=22.32, P<0.001) were more common in SLE with LSE. In addition, SLE with LSE had lower platelet counts [(140±67)×10 9/L vs (189±115)×10 9/L, t=-2.29, P=0.026] and higher positive rate of lupus anticoagulant (LA) (80.0% vs 23.6%, χ2=19.65, P<0.001), lower positive rate of anti-SSA antibodies (21.1% vs 60.0%, χ2=6.38, P=0.012). Left atrial enlargement (anteroposterior diameter of left atrium>40 mm) was more common in SLE patients with LSE (35.0% vs 5.4%, χ2=9.37, P=0.002), and anteroposterior diameter of left atrium [(37±7) mm vs (33±4) mm, t=2.15, P=0.043] were larger and left ventricular ejection fraction [(63±10)% vs (68±6)%, t=-2.41, P=0.019] was lower. The risk of SLE with LSE complicated with APS was 19 times compared with SLE without LSE [ OR (95% CI)=19.00 (4.43, 81.38), P<0.001]. Conclusion:SLE patients with LSE have increased risk of APS and cerebrovascular diseases. For patients with positive LA, the possibility of LSE should be alerted.
ABSTRACT
Resumen La endocarditis de Libman-Sacks es una forma de endocarditis no infecciosa, caracterizada por vegetaciones estériles que afectan principalmente las válvulas aórtica y mitral. Se asocia con enfermedades como lupus eritematoso sistémico y la manifestación más común es asintomática. Se relaciona con duración de la enfermedad, actividad lúpica y existencia de anticuerpos antifosfolipídicos. El diagnóstico se establece por ecocardiografía transtorácica y el definitivo por estudio de patología que demuestra vegetaciones en la autopsia o en muestras quirúrgicas. Se informa el caso de una paciente de 62 años que acudió por astenia, adinamia, ataque al estado general y disnea de medianos esfuerzos; en la ecocardiografía se encontraron vegetaciones. Se sometió a tratamiento quirúrgico y el diagnóstico se confirmó por estudio de patología.
Abstract Libman-Sacks endocarditis is a nonbacterial thrombotic endocarditis, characterized by the deposition of sterile platelet thrombi on heart valves, aortic and mitral. It is associated with systemic lupus erythematosus and the clinical manifestation is typically asymptomatic. It is associated with disease duration, lupus activity and antiphospholipid antibodies. The diagnosis is by echocardiography and the definitive diagnosis can be made pathologically by the demonstration of vegetations on autopsy or surgical speci mens. This paper reports the case of a 62-year old patient with asthenia, adynamia and dyspnea of medium efforts; the echocardiography showed vegetations and patient was underwent to surgical treatment with resection of vegetations, diagnosis was confirmed by pathology.
ABSTRACT
Libman-Sacks endocarditis (LSE) is a valvular heart disease that is associated with autoimmune diseases such as systemic lupus erythematosus and antiphospholipid syndrome (APS). Cases of LSE and APS associated with infection have been reported during the last several years. Herein, we present a patient who was suspected to have developed LSE and catastrophic APS during the treatment of her definite infective endocarditis, which was caused by Staphylococcus aureus, and the patient's condition was complicated with cerebral abscess, sensorineural hearing loss, endophthalmitis, renal infarction, splenic abscess, and septic arthritis.
Subject(s)
Humans , Abscess , Antiphospholipid Syndrome , Arthritis, Infectious , Autoimmune Diseases , Brain Abscess , Embolism , Endocarditis , Endophthalmitis , Hearing Loss, Sensorineural , Heart Valve Diseases , Lupus Erythematosus, Systemic , Splenic Infarction , Staphylococcus aureusABSTRACT
Relato de caso de paciente de 38 anos, feminina, com lúpus eritematoso sistêmico (LES) que apresentou evento tromboembólico arterial agudo em membro inferior direito. A investigação evidenciou a presença de anticorpos antifosfolípides e vegetação asséptica em válvula mitral, endocardite de Libman-Sacks (eLS). São discutidas as possíveis causas de eventos tromboembólicos arteriais no LES, com ênfase nas recomendações atuais para diagnóstico e tratamento da eLS.
Case report of a 38-year-old female patient with systemic lupus erythematosus (SLE) who presented an acute arterial thromboembolic event in the right lower limb. Investigation showed the presence of antiphospholipid antibodies and sterile vegetation in the mitral valve, Libman-Sacks endocarditis (LSE). Possible causes of thromboembolic events in SLE are discussed, with emphasis on current recommendations for diagnosis and treatment of LSE.
Subject(s)
Adult , Female , Humans , Antibodies, Antiphospholipid/immunology , Endocarditis/etiology , Endocarditis/immunology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/immunology , Thrombosis/etiology , Thrombosis/immunologyABSTRACT
Libman y Sacks describieron por primera vez en 1924 la presencia de lesiones valvulares en enfermos con lupus eritematoso. La endocarditis de Libman y Sacks se caracteriza por vegetaciones estériles valvulares de predominio en las válvulas mitral y aórtica. Su prevalencia es de 53 a 74 %. Se asocia con actividad lúpica y presencia de anticuerpos antifosfolípido. La ecocardiografía, en especial la transtorácica, es el estudio de elección para el diagnóstico y seguimiento, así como para evaluar el involucro valvular. El objetivo de este trabajo es presentar la imagen ecocardiográfica de un enfermo con lupus eritematoso activo portador de endocarditis de Libman-Sacks.
In 1924, Libman and Sacks described valvular lesions in patients with lupus erythematosus. Libman-Sacks valvular lesions are sterile fibrinous vegetations that preferentially develop at the left-sided heart valves. Reports of their prevalence range from 53 to 74%. Libman-Sacks endocarditis is associated with disease activity and antiphospholipid antibodies titer. Echocardiography is a useful tool for documenting valvular involvement and dysfunction as well as disease progression. Transesophageal echocardiography is superior to transthoracic echocardiography in detecting Libman-Sacks endocarditis. This report describes the echocardiographic image of Libman-Sacks endocarditis in a patient with active lupus erithematosus.
Subject(s)
Humans , Female , Middle Aged , Endocarditis , Lupus Erythematosus, Systemic/complications , Endocarditis/etiologyABSTRACT
Libman-Sacks endocarditis is characterized by noninfectious vegetations on the cardiac valves of patients with systemic lupus erythematosus (SLE). Valvular abnormalities are found in about one third of patients with primary anitiphospholipid syndrome. SLE patients, with antiphospholipid antibodies, have a higher prevalence of valvular involvement than those without these antibodies. A 29 year old man was referred for evaluation of severe orthopnea. He presented with the clinical features of multi-organ failure (heart, lung and kidney). His serological and immunological findings were typical of a SLE flare up. His level of anticardiolipin antibody (IgG) was highly increased and his lupus anticoagulant was positive. A blood culture revealed no growth of bacterial organism. Transthoracic and transesophageal echocardiography showed non-mobile, verrucous vegetations on the anterior leaflet of the mitral valve, with moderate mitral regurgitation.