Stress perfusion Cardiac Magnetic Resonance in Patients with Antiphospholipid Syndrome.

Maria Tektonidou,Sophie Mavrogeni,Petros Sfikakis,Genovefa Kolovou

doi:10.31138/mjr.29.2.99

Abstract

Background:Antiphospholipid syndrome (APS) is characterized by the combination of recurrent arterial and venous thrombotic events and detection of persistently elevated antiphospholipid antibody titers in the serum or plasma. APS clinical manifestations also include non-thrombotic complications from various organ systems, mainly the CNS, kidneys, and heart. Cardiac manifestations of APS include valvulopathy, myocardial infarction and angina (stable, unstable, and Pritzmental angina). A previously published case series of cardiac magnetic resonance (CMR) in patients with APS has revealed a high rate of asymptomatic myocardial necrosis and scarring, but the prevalence of myocardial ischemia identified as CMR perfusion defects prior to development of necrosis is unknown.Aims of the study:To detect CMR imaging markers of myocardial ischemia in APS patients without symptoms of cardiovascular disease (CVD).Methods:We will scan fifty APS patients without symptoms of CVD stress-perfusion CMR in a 1.5 Tesla tomographer, after intravenous infusion of adenosine and gadolinium. In addition to markers of cardiac anatomy and function, we will record imaging markers of ischemia and scarring, namely perfusion defects (PDs), and late gadolinium enhancement (LGE). We will perform parametrics using dedicated software in order to derive each patient’s myocardial perfusion reserve index (MPRI). Scans will be reviewed independently by two experienced reviewers, with evaluation of inter- and intra-observer reliability. Statistical hypotheses will be examined using Student’s test and Pearson’s correlation coefficient, or non-parametric equivalents (Kruskall-Wallis and Spearman) for continuous variables, and Fisher’s exact test for binary variables. Linear or logistic regression analyses will be used to investigate APS-related determinants of subclinical myocardial ischemia.Anticipated benefits:We expect to identify CMR imaging patterns characteristic of APS, which will allow proactive therapeutic interventions for primary prevention of CVD and guide further research into the pathogenesis of APS cardiac manifestations.

Highlights

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by venous, arterial, and/or small vessel thrombosis, pregnancy morbidity, and elevated levels of antiphospholipid antibodies, namely lupus anticoagulant (LAC), anticardiolipin antibodies, and/or anti-beta2-glycoprotein I antibodies.[1]
Any combination of thrombotic events is possible at variable intervals, and the syndrome can be either primary (PAPS) or associated with an underlying condition, most commonly systemic lupus erythematosus (SLE/APS)
Non-valvular heart disease manifesting as myocardial infarction (MI) has been diagnosed in 5.5% of APS and was the presenting manifestation in 2.8% of APS.[2]

Summary

Introduction

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by venous, arterial, and/or small vessel thrombosis, pregnancy morbidity, and elevated levels of antiphospholipid antibodies (aPL), namely lupus anticoagulant (LAC), anticardiolipin antibodies (aCL), and/or anti-beta2-glycoprotein I antibodies.[1]. The 5-year mortality in APS is 5.3%, with up to 40% of deaths related to serious thromboembolic events such as stroke, pulmonary embolism and acute myocardial infarction.[2] The most common type of cardiac involvement in APS is heart valve disease (HVD),[3,4] characterized by thickening and/or vegetation of the cardiac valves, as initially described by Libman and Sacks in patients with SLE.[5,6] The coexistence of antiphospholipid antibodies (aPL) with SLE is associated with a 3-fold greater risk of HVD.

Results

Conclusion