Cardiac Manifestations of Antiphospholipid Syndrome: Clinical Presentation, Role of Cardiac Imaging, and Treatment Strategies

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.

Left Atrial Myxoma and Antiphospholipid Syndrome—A Case Report

“…17 Cardiovascular manifestations of APS primarily include accelerated atherosclerosis, but other cardiac symptoms such as valvular disease thickening and vegetations may also be present. 5,18,19 Many cases of APS in the literature have reported cardiac thrombi mimicking myxoma 20 but only a few of them have described the coexistence of CM and APS which was found in our patient. Quintanilla et al have reported the case of right atrial myxoma in a female patient with left hemiplegia.…”

“…4 Antiphospholipid syndrome (APS) is manifested by the recurrent venous and arterial thrombotic events, recurrent fetal loss, and by the presence of specific circulating antiphospholipid antibodies (APA). 5 6 The coexistence of the CM and APS is rarely described in the literature . In this case report, we present a female patient with a left atrial mass, which was later found to be a CM and concomitant APS.…”

Risk Assessment and Antithrombotic Strategies in Antiphospholipid Antibody Carriers

“…Although less frequent than thromboembolism, CV disease (e.g., acute coronary syndrome, stroke, transient ischaemic attack) may sometimes represent the first manifestation of APS [15,57].…”

Anaesthetic considerations for patients with antiphospholipid syndrome undergoing non-cardiac surgery

“…First, valvular heart disease, which is the most common cardiac manifestation, represents a risk factor for postoperative arterial thromboembolism, such as peripheral arterial thrombosis and ischaemic stroke. 107 Thus, screening of valve lesions through transthoracic echocardiography (TTE) is necessary perioperatively, and detection or follow up of valve lesions through TTE or transesophageal echocardiography will be necessary for patients with APS. [108][109][110] Secondly, if patients complain of acute back pain, haematuria, or uncontrolled hypertension, then acute nephropathy due to renal artery thrombosis or thrombotic microangiopathy may be suspected; in these cases, doppler US or abdominal CT may be helpful for differential diagnosis, with the addition of urinalysis, renal function tests, and biopsies.…”