Cardiac manifestations in antiphospholipid syndrome - a brief review of the literature

Abstract

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.