Antiphospholipid syndrome and the heart: A case series and literature review

Denas, Gentian; Jose, S. Padayattil; Bracco, Alessia; Zoppellaro, Giacomo; Pengo, Vittorio

doi:10.1016/j.autrev.2014.11.003

Cited by 79 publications

(70 citation statements)

References 96 publications

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“…Vegetations on the mitral valve generally form on the atrial surface of leaflets [3,6]. Previous reports have also described "kissing lesions" located on the opposing lines of leaflet closure of both the mitral and the aortic valves such as in our case [3,5,6,8]. Decreased leaflet mobility and subvalvular thickening are rarely seen [3].…”

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confidence: 60%

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Primary cardiac involvement of anti-phospholipid syndrome misdiagnosed as infective endocarditis

Çağlar¹,

Bıyık²,

Işıksaçan³

et al. 2017

Arch Med Sci Civil Dis

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Anti-phospholipid syndrome (APS) is defined by thrombosis (venous, arterial, or small vessel) and/or pregnancy morbidity (recurrent miscarriage, fetal loss, or placental insufficiency) occurring in the presence of persistently positive anti-phospholipid antibodies (aPL), lupus anticoagulant (LAC) test, anti-cardiolipin antibodies (aCL), and anti-B2-glycoprotein I antibodies [1]. Anti-phospholipid syndrome can occur as an isolated condition or can be associated with connective tissue diseases, most commonly systemic lupus erythematosus (SLE). Herein we present a patient with aPL syndrome and cardiac involvement, misdiagnosed and treated as infective endocarditis.A 31-year-old woman was admitted to the neurology clinic because of weakness of the left hand. Her clinical history revealed mild weakness of bilateral hands lasting less than 2 h for about 5 years. Cranial contrast magnetic resonance imaging (MRI) showed atrophic gliotic changes and thin strip style gyral hyperintensity in T1 imaging at the superior sylvian fissure. Also gliotic localized atrophic changes were observed at the right posterior horn of the lateral ventricle. The patient was referred to the cardiology department for differential diagnosis. Initial physical examination and electrocardiography were evaluated as normal. Transthoracic and then transesophageal echocardiography revealed a hypodense fibrillated mobile 5 mm mass at the A2 scallop of the anterior mitral valve leaflet and moderate mitral regurgitation (Figures 1-3). Both MRI and transesophageal echocardiography (TEE) findings were consistent with endocarditis, so the patient was admitted to the cardiology department and consulted with rheumatology and infectious diseases. An empiric antibiotic regimen was started after blood culture sampling. Meanwhile her laboratory findings demonstrated all increased positive results of anti-b-2-glycoprotein-1 IgM and IgG, lupus anticoagulant, anti-cardiolipin IgM and IgG and decreased C4 levels. She had no fever and blood cultures were negative, so antibiotherapy was stopped at day 10, with the final diagnosis of primary APS and Libman-Sacks endocarditis. She was discharged with warfarin therapy.The common cardiac manifestations of APS include valvular involvement such as thickening, vegetations and dysfunction, coronary thrombosis, ventricular hypertrophy and dysfunction, intracardiac thrombi and pulmonary hypertension [2]. The most common echocardiographically

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confidence: 60%

“…The most common echocardiographically observed features are leaflet thickening and vegetations without serious valvular impairment [3,4]. Hemodynamically significant stenosis or regurgitation is uncommon [5]. The precise mechanism by which valves become deformed is not yet fully known [4,6].…”

mentioning

confidence: 99%

Primary cardiac involvement of anti-phospholipid syndrome misdiagnosed as infective endocarditis

Çağlar¹,

Bıyık²,

Işıksaçan³

et al. 2017

Arch Med Sci Civil Dis

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“…Known cardiac manifestations of APS include valvular disease,2 3 coronary artery disease and myocardial dysfunction presenting as dilated cardiomyopathy 4. Valvular disease is seen in about two-thirds of all patients.…”

Section: Discussionmentioning

confidence: 99%

Primary antiphospholipid syndrome, hypertrophic non-obstructive cardiomyopathy and hypotelorism

Kellermair¹,

Kammler

Laubichler

et al. 2016

BMJ Case Reports

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Antiphospholipid syndrome (APS) is an autoimmune disorder associated with arterial/venous thrombosis. Cardiac manifestations of APS include valve stenosis/insufficiency, coronary artery disease and myocardial dysfunction presenting as dilated cardiomyopathy. In the following report, we present the case of a man with primary APS, hypertrophic non-obstructive cardiomyopathy and hypotelorism-a combination that has not yet been reported in the literature.

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“…3,4 Finally, cases of accelerated atherosclerosis have also been described in patients with aPL or APS. [5][6][7] Table 1 lists the frequencies of the various different manifestations of APS, according to analysis of a large European cohort (1,000 patients). 8,9 Rarely, a devastating condition with rapid onset known as catastrophic antiphospholipid syndrome (CAPS) may be observed.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

A importância de reconhecer a síndrome antifosfolípide na medicina vascular

et al. 2017

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Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by recurrent arterial or venous thrombosis and/or gestational morbidity and by the presence of antiphospholipid antibodies. It can also cause other vascular manifestations such as microangiopathy, chronic arteriopathy and catastrophic APS (CAPS). Certain laboratory tests for the syndrome (for example, the lupus anticoagulant test) can be affected by the use of anticoagulant agents, making diagnosis more difficult. The pathophysiology of APS is complex, and several mechanisms of pathogenesis related to coagulation, endothelium, and platelets are discussed in this article. We conclude by discussing treatment of APS according to the presence and type of clinical manifestations, use of direct oral anticoagulants (DOAs), and perioperative management of patients with APS.Keywords: antiphospholipid syndrome; lupus anticoagulant; anticardiolipin antibodies; thrombosis; autoimmunity. ResumoA síndrome antifosfolipíde (SAF) é uma doença autoimune sistêmica caracterizada por trombose arterial ou venosa recorrente e/ou morbidade gestacional e pela presença dos anticorpos antifosfolipídeos, podendo apresentar outras manifestações vasculares, como microangiopatia, arteriopatia crônica e SAF catastrófica. Determinados testes laboratoriais para a síndrome (por exemplo, o anticoagulante lúpico) podem sofrer interferência do uso de medicações anticoagulantes, dificultando o diagnóstico. A fisiopatologia da SAF é complexa, sendo enumerados no texto diversos mecanismos patogênicos relacionados à coagulação, ao endotélio e às plaquetas. Por fim, discutimos o tratamento da SAF de acordo com a presença e o tipo de manifestações clínicas, o uso dos anticoagulantes orais diretos e o manejo perioperatório de pacientes com SAF.Palavras-chave: síndrome antifosfolípide; anticoagulante do lúpus; anticorpos anticardiolipina; trombose; autoimunidade.

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Antiphospholipid syndrome and the heart: A case series and literature review

Cited by 79 publications

References 96 publications

Primary cardiac involvement of anti-phospholipid syndrome misdiagnosed as infective endocarditis

Primary cardiac involvement of anti-phospholipid syndrome misdiagnosed as infective endocarditis

Primary antiphospholipid syndrome, hypertrophic non-obstructive cardiomyopathy and hypotelorism

A importância de reconhecer a síndrome antifosfolípide na medicina vascular

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