A Rare Case of Tricuspid Valve Libman-Sacks Endocarditis in a Pregnant Woman with Primary Antiphospholipid Syndrome

Abstract

Antiphospholipid Antibody Syndrome (APS) is a systemic autoimmune disease characterized by acquired hypercoagulability with the possible development of venous, arterial, and microvascular thrombosis. We report a rare case of Libman-Sacks tricuspid valve endocarditis in a 38-year-old pregnant woman at 15 weeks gestation with unknown primary antiphospholipid syndrome. During a routine cardiac examination and echocardiography performed for a previous episode of pleuropericarditis, a large, mobile mass with irregular edges was found at the level of the tricuspid valve. Three main differential diagnoses for intramyocardial mass were examined: tumor, infective endocarditis, and nonbacterial thrombotic endocarditis (NTBE). Cardiac magnetic resonance imaging (CMR) with contrast raised the suspicion of a thrombus. The woman was hospitalized urgently at the Cardiac Intensive Care Unit of the Federico II University Hospital, and anticoagulant and antiplatelet therapy were started. The thrombophilic screening performed and medical history confirmed the diagnosis of primary antibody syndrome (APS). A multidisciplinary consultation with obstetricians, cardiologists, anesthetists, and cardiac surgeons was required. The patient decided not to terminate the pregnancy despite the risk to her health and to undergo cardiac surgery during pregnancy. Histological examination confirmed the presence of nonbacterial thrombotic endocarditis. Weekly obstetric scans were performed after surgery to verify fetal well-being. An emergency cesarean section was performed at the 35th week of gestation due to repeated deceleration and abnormal short-term variability on c-CTG in a pregnancy complicated by fetal growth restriction and gestational hypertension. A newborn weighing 1290 g was born. She was hospitalized in Neonatal Intensive Care and discharged after two months; currently, she enjoys good health. The management of patients with antiphospholipid antibody syndrome has not yet been standardized, but there is a general consensus that patients who do not have thrombocytopenia, thromboembolic phenomena, or pregnancy should not undergo any treatment or should take only low doses of acetylsalicylic acid. In the presence of any of the above conditions, various treatment regimens have been used based on the severity and individuality of the case.

Keywords: Libman–Sacks endocarditis; antiphospholipid syndrome; pregnancy; primary antibody syndrome; thrombosis.

Figure 1

Standard (A) and 3D echocardiographic assessment (B) of right-side intracardiac mass.

Figure 2

Cardiac magnetic resonance depicting right non-vascularized intracardiac mass. CMR images: Cine SSFP short-axis image (A) showing the mass in the right ventricle (white arrow) with irregular borders. STIR T2-weighted (B) and FSE T1-weighted short-axis images (C) showing the lower signal intensity of the mass compared to the myocardium. Late post-contrast short-axis image (D) showing no-contrast uptake from the mass.

Figure 3

Histological specimen of the intracardiac mass revealing nonbacterial thrombotic endocarditis (i.e., Libman–Sacks endocarditis).