Abstract
Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence
of antiphospholipid antibodies, vascular thrombosis (venous, arterial, or small vessels),
and/or pregnancy morbidity. Diagnosis of APS is based on the presence of at least
one clinical criterion (thrombotic events or pregnancy morbidity) and at least one
of the laboratory criteria (persistently medium/high titer immunoglobulin G [IgG]/immunoglobulin
M [IgM] anticardiolipin antibodies, and/or medium/high titer IgG/IgM anti-β2-glycoprotein
I antibodies, and/or a positive lupus anticoagulant test), confirmed after repetition
at least 12 weeks apart. The clinical spectrum of APS encompasses additional (extracriteria)
clinical manifestations, including cardiac diseases. Heart involvement may become
evident as a consequence of direct (autoimmune-mediated) or indirect (thrombosis)
mechanisms, and include valve heart disease (vegetations and/or thickening associated
with functional abnormalities) and intracardiac thrombosis, coronary, and vascular
accelerated atherosclerosis, along with ischemic heart disease. APS can also cause
pulmonary arterial hypertension, left ventricular dysfunction, and heart failure.
This review describes the major cardiac manifestations of APS and illustrates the
role of cardiac imaging for diagnosing subclinical and overt heart involvement and
addressing management of these patients. The possible role of therapeutic strategies
in cardiac manifestations of APS is also discussed.
Keywords
antiphospholipid syndrome - atherosclerosis - thrombotic event - antiphospholipid
antibodies - echocardiography