Summary: | Antiphospholipid syndrome (APS) or Hughes syndrome represents a systemic
autoimmune disorder characterized by arterial and/or venous thrombosis,
multiple and recurrent fetal losses, accompanied by persistently elevated
levels of antiphospholipid antibodies (aPL). This syndrome is considered
primary if unassociated with any other connective tissue disease, or
secondary if it appears in association with other autoimmune disorders,
mainly systemic lupus erythematosus. Cardiac manifestations in APS are
integral part of the syndrome. aPL are involved in the pathogenesis of
pseudoinfective endocarditis (Libman Sacks) and other valvular manifestations
presented as their thickening and dysfunction. Intracardiac thrombi and
myxomas, pulmonary hypertension and left ventricular dysfunction are also
distinguishing features of APS. On the other hand, accelerated
atherosclerosis, proven in APS and also aPL mediated, is accountable for the
development of coronary and peripheral artery disease. This leads to higher
cardiovascular mortality rate in the population of patients with low
incidence of the traditional atherosclerosis risk factors. Furthermore,
recent studies implied that presence of certain aPL could be a risk factor
for a specific cardiac manifestation. Bearing all this in mind, early
diagnosis of cardiac manifestations, control and abolition of traditional
risk factors, as well as close cardiac follow-up of APS patients, are crucial
in reducing their cardiovascular mortality.
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