Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by arterial, venous, and/or small vessel thrombosis, pregnancy morbidity, and persistently elevated levels of antiphospholipid antibodies (aPL). Cardiovascular disease (CVD) in APS can present as heart valvular disease (HVD), macro-micro-coronary artery disease (CAD), myocardial dysfunction, cardiac thrombi, or pulmonary hypertension. Brain disease presents as stroke or transient ischemic attack (TIA) and less frequently as cerebral venous thrombosis, seizures, cognitive dysfunction, multiple sclerosis (MS)-like syndrome, or chorea. Infarcts and focal white matter hyperenhancement are the commonest brain (MRI) abnormalities, while myocardial ischemia/fibrosis, valvular stenosis/regurgitation, or cardiac thrombi are the main abnormalities detected by cardiovascular magnetic resonance. This review aims to present the existing evidence on brain/heart involvement and their interrelationship in APS and the role of brain/heart MRI in their evaluation. Embolic brain disease, due to HVD, CAD, and/or cardiac thrombus, or brain hypo-perfusion, due to myocardial dysfunction, are among the main brain/heart interactions in APS and they are considered determinants of morbidity and mortality. Currently, there is no evidence to support the use of combined brain/heart MRI in asymptomatic APS patients. Until more data will be available, this approach may be considered in APS patients at high risk for CVD/stroke, such as systemic lupus erythematosus with high-risk aPL profile or high scores in CVD risk prediction models; APS patients with HVD/thrombus, CAD, or heart failure; those with classic and non-criteria neurologic APS manifestations (seizures, cognitive dysfunction, MS-like syndrome); or with aggressive multi-organ disease. Key Points • Cardiovascular disease (CVD) in antiphospholipid syndrome (APS) can present as heart valvular disease (HVD), macro-micro-coronary artery disease (CAD), myocardial dysfunction, cardiac thrombi, or pulmonary hypertension. • Brain disease presents as stroke or transient ischemic attack (TIA), and less frequently as cerebral venous thrombosis, seizures, cognitive dysfunction, and multiple sclerosis (MS). • A combined brain/heart MRI may be considered in APS patients at high risk for CVD/stroke, such as systemic lupus erythematosus with high-risk aPL profile or high scores in CVD risks; APS patients with HVD/thrombus, CAD, or heart failure; those with classic and non-criteria neurologic APS manifestations (seizures, cognitive dysfunction, MS-like syndrome); or with aggressive multi-organ disease.

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