Clinical Features of Antiphospholipid Syndrome with Intracardiac Mass.

Abstract

Antiphospholipid syndrome (APS), a disorder characterized by the presence of antiphospholipid antibodies, is commonly associated with thrombotic events and pregnancy complications. Although cardiac involvement of APS is very common, intracardiac thrombus is rare and easily misdiagnosed. In order to reduce missed diagnosis and misdiagnosis, we investigated the clinical features of APS with intracardiac mass by summarizing 50 cases (1 newly presented case and 49 additional cases collected from PubMed from 1985 to the present). There were 10 males and 40 females, with ages ranging from 8 to 75 years (median age 35.5). Intracardiac masses were distributed in four cardiac chambers. Mass size ranged from a diameter of 0.5 to 7.1 cm. Clinical manifestations were heterogeneous, including dyspnea, fever, hemiparesis, limb ischemia, and other nonspecific symptoms. In 41 cases with available pathology results, 33 cases were confirmed as thrombus, 2 cases as myxoma, 3 cases as non-bacterial endocarditis, 2 cases as fibrous tissue, and 1 case as inflammatory necrosis. Among 41 cases, 18 cases were suspected of primary cardiac tumors preoperatively, while pathological examination revealed none was tumor. APS patients with intracardiac masses are extremely rare, mostly seen in young or middle-aged people, and they present with a variety of clinical manifestations. Most masses disappear following medical treatment. APS can be accompanied by cardiac myxomas. APS should be promptly investigated in young patients presenting with thrombotic events without any underlying risk factors.